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  • 1. COLLEGE OF MEDICINE CURRICULUM VITAE Richard J.H. Smith, MD I. EDUCATIONAL AND PROFESSIONAL HISTORY A. Education 1974 BA Rice University (Biochemistry) 1977 MD Baylor College of Medicine 1977-78 Internship University of Texas Health Science Center Houston, Texas 1978-79 General Surgery University of Texas Health Science Center Residency Houston, Texas 1979-82 Otolaryngology Baylor College of Medicine Residency Houston, Texas 1982-83 Fellowship The Hospital for Sick Children Pediatric Otolaryngology, London, England 1990-91 Research Sabbatical Medical Research Council Institute of Hearing Research Nottingham, England Certification: Diplomate, American Association of Otolaryngology-HNS September, 1982 Licensure: 1. Texas June, 1977 Permanent E8924 2. Iowa February, 1997 Permanent 27940 B. Academic Appointments 1983-88 Assistant Professor Department of Otolaryngology Baylor College of Medicine 1988-90 Associate Professor Department of Otolaryngology Baylor College of Medicine 1990- Professor Department of Otolaryngology-HNS University of Iowa College of Medicine 1996- Professor Interdisciplinary Genetics PhD Program University of Iowa Graduate School 1999- Sterba Hearing University of Iowa College of Medicine Research Professor C. Honors and Awards 1974 Phi Beta Kappa 1974 Phi Lambda Upsilon 1974 Queeny Foundation Academic Scholarship 1975 A.B. Cohn Academic Scholarship
  • 2. Smith RJH 2 1982 Harriet Cunningham Citation: Awarded for meritorious scientific writing in recognition of articles of exceptional scientific quality. 1985 Texas Department of Health: Certificate of Appreciation in Recognition of Meritorious Service to the State of Texas 1991 Barry J. Anson Outstanding Teacher Award 1992 Shirley Baron Award: Outstanding Research 1994 The Best Doctors in America, 1994. South Carolina: Woodward/White Inc. 1995 Seymour Cohen Award: Outstanding Clinical Research 1995 Barry J. Anson Outstanding Teacher Award 1996 Stowell-Orbison Award (2nd): United States and Canadian Academy of Pathology (Outstanding Research) 1997 Alberta Heritage Foundation Visiting Professor in Medical Genetics 1999 Collegium ORLAS 1999 William Potsic Award (1st): Outstanding Basic Science Research, ASPO 1999 The Best Doctors in America, 1999. South Carolina: Woodward/White Inc. 2001 Charles Ferguson Award (1st): Outstanding Clinical Research, ASPO 2002 William Potsic Award (1st): Outstanding Basic Science Research, ASPO 2002 The Best Doctors in America, 2002. South Carolina: Woodward/White Inc. 2002 State of Iowa Quality of Care Award (Given annually to two doctors in Iowa for integrity and professionalism, and being chosen as a role model for community leadership.) 2003 Honored Guest – Canadian Society of Otolaryngology – Head and Neck Surgery 2003 Robin Cotton and Cynthia Fitton Award and Honored Guest, American Academy of Otolaryngology – Head and Neck Surgery 2003 Robert Ruben Award and Honored Guest – Society for Ear, Nose and Throat Advances in Children (SENTAC) 2004 The Best Doctors in America, 2004. South Carolina: Woodward/White Inc. I. TEACHING AT THE UNIVERSITY OF IOWA A. Teaching Assignments 1. Classroom Teaching 1991 Functional Endoscopic Sinus Surgery; Course Director; May, 1991 nd 1992 2 Annual Functional Endoscopic Sinus Surgery Course - Advanced Techniques and Complications; Course Director; May, 1992 1993 3rd Annual Functional Endoscopic Sinus Surgery Course; Course Director; May, 1993 1994 4th Annual Functional Endoscopic Sinus Surgery Course; Course Director; May, 1994 1995 5th Annual Functional Endoscopic Sinus Surgery Course; Course Director, May, 1995 1995 Medical Ethics M2 Course, 1 hour/week, September - December 1995 Medical Genetics M1 Course – Small Group Session 1996 6th Annual Functional Endoscopic Sinus Surgery Course; Course Director, May, 1996 1996 Medical Genetics M1 Course – Small Group Session 1997 7th Annual Functional Endoscopic Sinus Surgery Course; Course Director, June, 1997
  • 3. Smith RJH 3 1997 Human Genetics, Genetics PhD Program 1997 Medical Genetics M1 Course – Small Group Session 1998 8th Annual Functional Endoscopic Sinus Surgery Course; Course Director, June, 1998 1998 Human Genetics, Genetics PhD Program 1998 Medical Genetics M1 Course – Small Group Session 1999 9th Annual Functional Endoscopic Sinus Surgery Course; Course Director, June, 1999 1999 Human Genetics, Genetics PhD Program 1999 Medical Genetics M1 Course – Small Group Session 2000 Genetics Seminar Course 127:200, Genetics PhD Program 2000 Human Genetics, Genetics PhD Program 2000 Medical Genetics M1 Course – Small Group Session 2001 11th Annual Functional Endoscopic Sinus Surgery Course; Course Director, June, 2001 2001 Human Genetics, Genetics PhD Program 2001 Medical Genetics M1 Course – Small Group Session 2002 1st Iowa Airway Course, April, 2002 2002 12th Annual Functional Endoscopic Sinus Surgery Course; Course Director, June, 2002 2002 Human Genetics, Genetics PhD Program 2002 Medical Genetics M1 Course – Small Group Session 2003 2nd Iowa Airway Course, April, 2003 2003 13th Annual Functional Endoscopic Sinus Surgery Course; Course Director, June, 2003 2003 Human Genetics, Genetics PhD Program 2003 Medical Genetics M1 Course – Small Group Session 2. Clinical Teaching 1990- Pediatric Rounds 1 hour/week, 52 weeks/year 1990- Clinical Conferences 3-4 days/year 1990- Neonatology Rounds 2 hours/year B. Graduate Students Supervised 1. Thesis Advisor Norio Kasai - PhD candidate, Okayama University. Thesis: A gene for fluctuating, progressive autosomal dominant non-syndromic hearing loss, DFNA16, maps to chromosome 2q23-24; Genomic structures of SCN2A and SCN3A - Candidate genes for deafness at the DFNA16 locus. PhD awarded through Okayama University, December 22, 2000. Tao Yang - PhD candidate, Interdepartmental PhD Program in Genetics. Thesis advisor. Wenjie Chen - PhD candidate, Interdepartmental PhD Program in Genetics. Thesis advisor.
  • 4. Smith RJH 4 Hela Azaiez - PhD candidate, University of Sfax, Tunisia. Thesis advisor. 2. Thesis Committee Bryan Bjork - PhD (2000) Interdepartmental PhD Program in Genetics. Thesis committee. Doug Dylla - PhD candidate, Interdepartmental PhD Program in Genetics. Thesis committee. Paul Grandgenett - PhD candidate, Interdepartmental PhD Program in Genetics. Thesis committee. Xie Hehuang - PhD candidate, Interdepartmental PhD Program in Genetics. Thesis committee. Peter Jezewski - PhD candidate, Interdepartmental PhD Program in Genetics. Thesis committee. Daryl Scott - PhD (1998) Interdepartmental PhD Program in Genetics. Thesis committee. Rebecca Schultz - PhD candidate, Interdepartmental PhD Program in Genetics. Thesis committee. Theresa Zucchero - PhD candidate, Interdepartmental PhD Program in Genetics. Thesis committee. Ryan Kavlie - PhD candidate, Interdepartmental PhD Program in Genetics. Thesis committee. Sokol Todi – PhD candidate, Neuroscience Graduate Program. Thesis committee. 3. Pediatric Otolaryngology Fellows Trained Mike Biavati,MD – July, 1992 – June, 1993 Nancy Bauman, MD – July, 1993 – June, 1994 John Bent, MD – July, 1994 – June, 1995 Leila Mankarious, MD – July, 1995 – June, 1996 John Greinwald, MD – July, 1996 – June, 1998 Jose Manaligod, MD – July, 1997 – June, 1999 Glenn Green, MD – July, 1998 – June, 2000 Stephen Hone, MD – July, 2000 – June, 2002
  • 5. Smith RJH 5 Chantal Giguere, MD – July, 2000 – June, 2002 Ben Cable, MD – July, 2001 – June, 2003 Murad Hussein, MD – July, 2002 – June, 2004 D. Instructional Web Pages Hereditary Hearing Loss Homepage (http://dnalab-www.uia.ac.be/dnalab/hhh/) II. SCHOLARSHIP A. Bibliography Papers Published or In Press 1. Jarratt M, Smith, RJH, Knox J. Therapy of herpes simplex infection. Int J Derm 18(5):357-361, 1979. 2. Igarashi M, O-Uchi T, Smith RJH, Stout B. Inner ear pathology in deaf mutism. J Otolaryngol 10(2):99-108, 1981. 3. Sessions RB, Lehane D, Smith RJH, Bryan N, Suen J. Intra-arterial cisplatin- treatment of adenoid cystic carcinoma. Arch Otolyngol 108(4):221-224, 1982. 4. Smith RJH, Sessions RB, Bean S. Benign mucous membrane pemphigoid. Ann Oto Rhino Laryngol 91(2):142-144, 1982. 5. Smith RJH. Creating alaryngeal speech. Ear Nose Throat J 61(4):18-27, 1982. 6. Smith RJH, Sessions RB. The occult primary. Texas Med 78(10):55-58, 1982. 7. Smith RJH, Catlin FI. Congenital anomalies of the larynx. Am J Dis Child 138(1):35-40, 1984. 8. Smith RJH, Smith MCF, Glossop LP, Bailey CM, Evans JHG. Congenital vascular anomalies causing tracheo-esophageal compression. Arch Otolaryngol 110(2):82-87, 1984. 9. Smith RJH, Catlin FI. Pediatric Otolaryngology. Watch 30(2):20-24, 1984. 10. Smith RJH, Evans JNG. Head and neck manifestations of histiocytosis-x.Laryngoscope 94(3):395-399, 1984. 11. Spankus E, Flint P, Smith RJH, Miller RH. Craniocervical necrotizing fasciitis. Otolaryngol Head Neck Surg 92(3):261-266, 1984. 12. Glossop LP, Smith RJH, Evans JNG. Posterior laryngeal cleft: An analysis of 10 cases. Int J Ped Otolaryngol 7(2):133-143, 1984.
  • 6. Smith RJH 6 13. Smith MCF, Smith RJH, Bailey CM. Primary cervical neuroblastoma in neonates. J Laryngol Otol XCIX(2):209-214, 1985. 14. Spires JR, Smith RJH, Catlin FI. Brain abscesses in children. Otolaryngol Head Neck Surg 93(4):468-473, 1985. 15. Smith RJH. Classical influence on aural anatomy and physiology. Sydsvensk medicinhistoriska 12:69-81, 1985. 16. Wright CL, Smith RJH, Katz CD, Atkins JH. Benign parotid diseases of childhood. Laryngoscope 95(8):91 1985. 17. Smith RJH, Coombe WT. Aero-digestive compromise due to congenital vascular anomalies. Contemporary Pediatrics 2(10):94-106, 1985. 18. Franklin DJ, Smith RJH, Person DA, Pflugfelder SC. Sjogren's syndrome in children. Otolaryngol Head Neck Surg 94(2):230-234, 1986. 19. White AK, Smith RJH, Bigler CR, Brooke WF, Schauer, P.R. Head and neck manifestations of neurofibromatosis. Laryngoscope 96(7):732-737, 1986. 20. Spires JR, Smith RJH. Bacterial infections of the orbital and periorbital soft-tissues in children. Laryngoscope 96(7):763-767, 1986. 21. Spires JR, Smith RJH. Cat-scratch disease. Otolaryngol Head Neck Surg 94(5):622-627, 1986. 22. White AK, Smith RJH. Thyroid nodules in children. Otolaryngol Head Neck Surg 95(1) :70-75, 1986. 23. Franklin DJ, Smith RJH, Catlin FI, Helfrick JF, Foster JH. Temporo-mandibular joint dysfunction in infancy. Int J Ped Otolaryngol 12(1):99-104, 1986. 24. Kearns DB, Smith RJH, Pitcock JK. Burkitt's lymphoma. Int J Ped Otolaryngol 12(1):73-84, 1986. 25. Smith RJH. Editorial comment. Head Neck Surg 8(3):1986. 26. Weber RS, Kearns D, Smith RJH. Split calvarium cranioplasty. Arch Otolaryngol Head Neck Surg 113(1):84-89, 1987. 27. Cepero R, Smith RJH, Catlin FI, Bressler KL, Furuta GT, Shandera KC. Cystic fibrosis - an otolaryngologic perspective. Otolaryngol Head Neck Surg 97(4):356-360, 1987. 28. Smith RJH. Laryngotracheal stenosis. Head Neck Surg 10(1):38-47, 1987. 29. Catlin FI, Smith RJH. Acquired Subglottic Stenosis in Children. Ann Otol Rhinol Laryngol 96(5):488-492, 1987.
  • 7. Smith RJH 7 30. Daiger SP, Bertin TK, Smith RJH, Pelias MZ. Linkage of Usher's syndrome to CC on human chromosome 4. Human Gene Mapping 9, Cytogenet Cell Genet 46(1-4):602, 1987. 31. Brock ME, Smith RJH, Parey SE, Mobley DL. Lymphangioma - an otolaryngologic perspective. Int J Ped Otolaryngol 14:133-140,1987. 32. Robinson LN, Smith RJH, Rightmire Z, Torpy JM, Fernbach, DJ. Head and neck malignancies in children - an age-incidence study. Laryngoscope 98(1):11-13, 1988. 33. Smith RJH. Adenoidal hypertrophy and facial deformity. The collected letters of the international correspondence society of otolaryngologists. Series 23:16. 34. Smith RJH. Choanal atresia. The collected letters of the international correspondence society of otolaryngologists. Series 23:16. 35. Diaz E, Adams J, Hawkins H, Smith RJH. Tracheal agenesis: a case report & literature review. Arch Otolaryngol 115(6):741-5, 1989. 36. Smith RJH, Holcomb JD, Daiger SP, Caskey CT, Pelias MZ, Alford BR, Fontenot DD, Hejtmancik JF. Exclusion of Usher's syndrome gene from much of chromosome 4. Cytogenet Cell Genet 50(2-3):102-106, 1989. 37. Smith RJH. A DNA linkage study of Usher's syndrome excluding much of chromosome 4. Laryngoscope 99(9):940-9, 1989. 38. Dailey ME, O'Laughlin MP, Smith RJH. Airway compression secondary to left atrial enlargement and increased pulmonary artery pressure. Int J Otolaryngol 19:33-44, 1990. 39. Ohlms LA, Edwards MS, Mason EO, Igarashi M, Alford BR, Smith RJH. Recurrent meningitis and Mondini dysplasia. Arch Otolaryngol 116(5):608-12, 1990. 40. Kimberling WJ, Weston MD, Moller C, Davenport SLH, Shugart YY, Priluck IA, Martini A, Smith RJH. Localization of Usher syndrome type II to chromosome lq. Genomics 7: 245-49, 1990. 41. Connolly PK, Stout GG, Williams ST, Jorgensen S, Smith RJH. Oral habilitation of the child with no response on brainstem audiometry. Pediatrics 86(2):217-220, 1990. 42. DuBois JJ, Pokorny WJ, Harberg FJ, Smith RJH. Current management of laryngeal and laryngotracheoesophageal clefts. J Pediatr Surg 25(8):855-60, 1990. 43. Eicher SA, Coker NJ, Alford BR, Igarashi M, Smith RJH. A comparative study of the fallopian canal at the meatal foramen and labyrinthine segment in young children and adults. Arch Otolaryngol 116:1030-1035, 1990.
  • 8. Smith RJH 8 44. Pelias MZ, Smith RJH, Daiger SP, Hejtmancik JF. Usher syndrome in Louisiana. Proceedings of the Sixth World Congress of the International Retinitis Pigmentosa Association, 1990. 45. Kimberling WJ, Weston MD, Dahl PS, Shugart YY, Kenyon JB, Overbeck L, Moller C, Martini A, Smith RJH, Milani M. Progress in the localization of the Usher syndrome genes. Proceedings of the Sixth World Congress of the International Retinitinitis Pigmentosa Association, l990. 46. Smith RJH, Catlin FI. Laryngotracheal stenosis: A five-year study. Head Neck Surg 13(2):140-144, 1991. 47. Pieke-Dahl S, Weston MD, Kimberling WJ, Kenyon JB, Shugart YY, Smith RJH. Report on attempts to localize Usher syndrome type 1 by linkage analysis to selected candidate regions. Ann N Y Acad Sci 630:298-300, 1991. 48. Weston MD, Kimberling WJ, Möller CG, Pieke-Dahl S, Smith RJH, Martini A, Milani M. A progress report on the localization of Usher syndrome type II to chromosome 1q. Ann N Y Acad Sci 630:284-287, 1991. 49. Kimberling WJ, Weston M, Pieke-Dahl S, Kenyon JB, Shugart YY, Möller, C, Davenport SL, Martini A, Milani M, Smith RJ. Genetic studies of Usher syndrome. Ann N Y Acad Sci 630:167-175, 1991. 50. Smith RJH, Ohlms LA, Alford BR, Edwards MS. Mondini dysplasia is not associated with meningitis and cerebrospinal fluid fistula. Archives Otolaryngol 117:931-932, 1991. 51. Smith RJH, Snyderman, NL. Fibrosarcoma of the infratemporal fossa in an eight year old. Controversies. Head Neck 13(2):156-159, 1991. 52. Smith RJH, Synderman, NL. Distal tracheal compression. Controversies. Head Neck 13(3):251-254, 1991. 53. Steel KP, Smith RJH. Normal hearing in splotch (Sp/+), the mouse homologue of Waardenburg syndrome type I. Nature Genet 2:75-79, 1992. 54. Connolly PK, Jerger S, Williamson WD, Smith RJH, Demmler G. Evaluation of central auditory function in children with asymptomatic congenital cytomegalovirus. Amer J Otol 13:185-193, 1992. 55. Smith RJH, Pelias MZ, Daiger SP, Keats B, Kimberling WJ, Hejtmancik JF. Clinical and genetic heterogeneity within the Acadian Usher population. Amer J Human Genet 43:964-969, 1992. 56. Blumer JR, Bauman NM, Kearns DB, Smith RJH. Distal tracheal stenosis in neonates and infants. Otolaryngology Head Neck Surg 107:583-590, 1992.
  • 9. Smith RJH 9 57. Smith RJH, Steel KP, Barkway C, Soucek S, Michaels L. A histological study of non-morphogenetic forms of hereditary hearing impairment. Arch Otolaryngol 118:1085-1094, 1992. 58. Smith RJH, Zimmerman B, Connolly PK, Jerger SW, Yelich A. Screening audiometry using the high risk register in a level III nursery. Arch Otolaryngol 118:1306-1311, 1992. 59. Robinson MF, Furst EJ, Nunziata V, Brandi ML, Ferrer JP, Martins Bugalh MJG, diGiovanni G, Smith RJH, Donovan DT, Alford BR, Hejtmancik JF, Colantuoni V, Quadri L, Limbert E, Halperin I, Vilardell E, Gagel RF. The multiple endocrine neoplasia type 2/cutaneous lichen amyloidosis syndrome - clinical feature of a new variant. Calcium Regulation, Bone Metabolism - Basic and Clinical Aspects 11, 1992. 60. Keats BJB, Todorov AA, Atwood LD, Pelias MZ, Hejtmancik JF, Kimberling WJ, Leppert M, Lewis RA. Smith RJH. Linkage studies of Usher syndrome type I: exclusion results from the Usher syndrome consortium. Genomics 14(3):707-714, 1992. 61. Kimberling W, Smith RJH. Gene Mapping of the Usher Syndromes. Clinics N Amer 25(5):923-934, 1992. 62. Kumar S, Kimberling WJ, Kenyon JB, Smith RJH, Marres HAM, Cremers CWRJ. Autosomal dominant branchio-oto-renal syndrome - localization of a disease gene to chromosome 8q by linkage in a Dutch family. Human Molecular Genet 1(7):491-496, 1992. 63. Smith RJH, Lee EC, Kimberling WJ, Daiger SP, Pelias MZ, Keats BJB, Jay M, Bird A, Reardon W, Guest M, Ayyagari R, Hejtmancik JF: Localization of two genes for Usher syndrome type I to chromosome 11. Genomics 14(4):995-1002, 1992. 64. Kimberling WJ, Möller CG, Davenport S, Priluck IA, Beighton PH, Reardon W, Kenyon JB, Grunkmeyer JA, Pieke Dahl S, Overbeck LD, Blackwood DJ, Brower AM, Hoover DM, Rowland P, Smith RJH. Linkage of Usher syndrome type I to the long arm of chromosome 11. Genomics 14(4):988-994, 1992. 65. Smith RJH, Coppage KB, Ankerstjerne JKB, Capper DT, Kumar S, Kenyon J, Kimberling WJ. Localization of the gene for branchiootorenal syndrome to chromosome 8q. Genomics 14(4):841-844, 1992. 66. Smith RJH, Synderman NL. Juvenile nasopharyngeal angiofibroma. Controversies. Head Neck 14(1): 67-71, 1992. 67. Smith RJH, Snyderman NL. Fibrous dysplasia. Controversies. Head Neck 14(6):510-512, 1992.
  • 10. Smith RJH 10 68. Wackym PA, Simpson TA, Gantz BJ, Smith RJH. Polymerase chain reaction amplification of DNA from archival celloidin-embedded human temporal bone sections. Laryngoscope 103(6):583-588, 1993. 69. Gartlan MG, Davies J, Smith RJH. Congenital Oral Synechiae. Ann Otol Laryngol Rhinol 102(3):186-197, 1993. 70. Walker WP, Smith RJH, Cohen MB. Fine needle aspiration biopsy of subcutaneous fat necrosis of the newborn. Diag Cytopathol 9(3):329-332,1993. 71. Gartlan MG, Peterson KL, Hoffman HT, Luschei ES, Smith RJH. The bipolar hooked-wire electromyographic technique in the evaluation of pediatric vocal cord paralysis. Ann Otol Laryngol Rhinol 102:695-700, 1993. 72. Trigg ME, Menezes AH, Giller R, Lanza L, Smith RJH, Yutako S, Peters C, Altman A. Operative treatment of disseminated aspergillosis of the lung and brain following a marrow transplant: a case report. Bone Marrow Transplantation 11:493-496, 1993. 73. Smith RJH, Snyderman NL. Neonatal vocal cord paralysis. Controversies. Head Neck 15(2):169-172, 1993. 74. Endres D, Bauman NM, Burke D, Smith RJH. Acyclovir in the treatment of recurrent respiratory papillomatosis. Ann Otol Laryngol Rhinol 103:301-305, 1994. 75. Smith RJH, Berlin C, Hejtmancik JF, Keats B, Kimberling WJ, Lewis RA, Möller CG, Pelias MZ, Tranebjærg L. Clinical diagnosis of the Usher syndromes. Am J Med Genet 50:32-38, 1994. 76. Bauman NM, Sandler AD, Schmidt C, Maher JW, Smith RJH. Reflex laryngospasm induced by stimulation of distal esophageal afferents. Laryngoscope 104:209-214, 1994. 77. Bittleman DB, Smith RJH, Weiler JM. Abnormal movement of the arytenoid region during exercise presenting as exercise-induced asthma in an adolescent athlete. Chest 106:615-616, 1994. 78. Ni L, Wagner MJ, Kimberling, WJ, Pembrey ME, Grundfast KM, Kumar S, Daiger SP, Wells DE, Johnson K, Smith RJH. Refined Localization of the branchiootorenal syndrome gene by linkage and haplotype analysis. Amer J Med Genet 51:176-184, 1994. 79. Smith RJH, Neville MB, Bauman NM. Interarytenoid notch height relative to the vocal folds: A pilot study. Ann Oto Laryngol Rhinol 103:753-757, 1994. 80. Burgess RC, Michaels L, Bale JF, Smith RJH. Polymerase chain reaction amplification of Herpes Simplex Viral DNA from the geniculate ganglion of a patient with Bell's palsy. Ann Oto Laryngol Rhinol 103:775-779, 1994.
  • 11. Smith RJH 11 81. Mankarious LA, Bauman NM, Sandler AD, Maher JW, McCulloch TM, Smith RJH. Esophagolaryngeal adductor reflex induced by electrical stimulation of the distal esophageal mucosa of dogs. Transactions, Am Broncho Esophagol Assoc 36-40, 1994. 82. Coppage K, Smith RJH. Branchio-Oto-Renal syndrome. J Amer Acad Audiol 6:103-110, 1995. 83. Simpson T, Smith RJH. Amplification of mitochondrial DNA from archival temporal bone specimens. Laryngoscope 105(1):28-34, 1995. 84. Smith RJH. Decreased incidence of scoliosis in hearing impaired children: implications for a neurological basis for idiopathic scoliosis. Spine 20:7, 1995. 85. Biavati MJ, Wood WE, Kearns DB, Smith RJH. One-stage repair of congenital laryngeal webs. Otolaryngol Head Neck Surg 112:447-452, 1995. 86. Angeli SI, Alcalde J, Hoffman HT, Smith RJH. Langerhans' cell histiocytosis of the head and neck in children. Ann Otol Laryngol Rhinol 104(3):173-180, 1995. 87. Laurenzo JF, Canady JW, Zimmerman MB, Smith RJH. Craniofacial growth in rabbits. Effects of midfacial surgical trauma and rigid plate fixation. Arch Otolaryngol Head Neck Surg 121:556-561, 1995. 88. Smith RJH. Management of inverted papilloma. Controversies. Head Neck 17:148-151, 1995. 89. Smith RJH. Chronic sinusitis in a child. Controversies. Head Neck 17:252-257, 1995. 90. Pinkston DR, Schubkegel AJ, Zimmerman MB, Smith RJH. The effects of sinus surgery on midfacial growth in the rabbit. Amer J Rhinol 9(2):115-124, 1995. 91. Palmer PM, Dutton JM, McCulloch TM, Smith RJH. Trends in the use of tracheotomy in the pediatric patient: The Iowa experience. Head Neck 17:328-333, 1995. 92. Chen AH, Ni L, Fukushima K, Marietta J, O'Neill M, Coucke P, Willems P, Smith RJH. Linkage of a gene for dominant non-syndromic deafness to chromosome 19. Human Mole Genet 4(6):1073-1076, 1995. 93. Smith RJH, Bauman NM, Bent JP, Kramer M, Smits WL, Ahrens RC. Exercise- induced laryngomalacia. Ann Oto Laryngol Rhinol 104(7):537-541, 1995. 94. Dutton JM, Palmer PM, McCulloch TM, Smith RJH. Mortality in the pediatric patient with tracheostomy. Head Neck 17:403-408, 1995. 95. Mathews K, Rapisarda D, Bailey HL, Murray JC, Schelper R, Smith R. Phenotypic and pathologic evaluation of the MYD mouse. A candidate model for facioscapulohumeral dystrophy. J Neuropathol Exp Neurol 54:601-606, 1995.
  • 12. Smith RJH 12 96. Fukushima K, Ramesh A, Srisailapathy CRS, Ni L, Chen A, O’Neill M, Camp GV, Coucke P, Smith SD, Kenyon JB, Jain P, Wilcox ER, Zbar RIS, Smith RJH. Consanguineous nuclear families used to identify a new locus for recessive non- syndromic hearing loss on 14q. Human Mole Genet 4(9):1643-1648, 1995. 97. Chen AH, Francis M, Ni L, Cremers CWRJ, Kimberling WJ, Sato Y, Phelps PD, Bellman SC, Wagner MJ, Pembrey M, Smith RJH. Phenotypic manifestations of branchiootorenal syndrome. Am J Med Genet 58:365-370, 1995. 98. Lesperance MM, Hall JW, Bess FH, Fukushima K, Jain PK, Ploplis B, San Agustin TB, Skarka H, Smith RJH, Wills M, Wilcox ER. A gene for autosomal dominant nonsyndromic hereditary hearing impairment maps to 4p16.3. Hum Mole Genet 4:1967-1972, 1995. 99. Van Camp G, Coucke P, Balemans W, Van Velzen D, Van de Bilt C, Van Laer L, Smith RJH, Fukushima K, Padberg GW, Frants RR, Van de Heyning P, Smith SD, Huizing EH, Willems PJ. Localization of a gene for non-syndromic hearing loss (DFNA5) to chromosome 7p15. Hum Mole Genet 4(11):2159-2163, 1995. 100. Fukushima K, Arabandi R, Srisailapathy CRS, Ni L, Wayne S, O’Neill ME, Van Camp G, Coucke P, Jain P, Wilcox ER, Smith SD, Kenyon JB, Zbar RIS, Smith RJH. An autosomal recessive non-syndromic form of sensorineural hearing loss maps to 3p-DFNB6. Genome Research 5:305-308, 1995. 101. Jain PK, Fukushima K, Deshmukh D, Arabandi R, Thomas E, Kumar S, Lalwani AK, Ploplis B, Skarka H, Srisailapathy CRS, Wayne S, Zbar RIS, Verma IC, Smith RJH, Wilcox ER. A human recessive neurosensory nonsyndromic hearing impairment locus is a potential homologue of the murine deafness (dn) locus. Hum Mole Genet 4:2391-4, 1995. 102. Ayyagari R, Smith RJH, Pelias MZ, Hejtmancik JF. Fine mapping of the Usher syndrome type 1C to chromosome 11p14 and identification of flanking markers by haplotype analysis. Mole Vision 1:1995. 103. Sawin PD, Muhonen MG, Sato Y, Smith RJH. Aneurysmal bone cyst of the temporal bone presenting as hearing loss in a child. Inter J Ped Otorhinolaryngol 33:275-284, 1995. 104. Zbar RIS, Smith RJH. Vocal fold paralysis in infants. Otolaryngol Head Neck Surg 114:18-21, 1996. 105. Bauman NM, Sandler AD, Smith RJH. Respiratory symptoms of gastroesophageal reflux disease in pediatric patients. Ann Oto Laryngol Rhinol 105:23-32, 1996. 106. Smith RJH. Infratemporal fossa mass in a pediatric patient. Controversies. Head Neck 18(2):188-91, 1996. 107. Bent JP, Kim JW, Wilson JS, Miller DA, Bauman NM, Smith RJH. Pediatric exercise-induced laryngomalacia. Ann Oto Laryngol Rhinol 105:169-75, 1996.
  • 13. Smith RJH 13 108. Bauman NM, Oyos TL, Murray DJ, Kao SCS, Biavati MJ, Smith RJH. Post- operative care following single stage laryngotracheoplasty. Ann Oto Laryngol Rhinol 105:317-22, 1996. 109. Zbar RIS, Chen AH, Behrendt DM, Bell EF, Smith RJH. The incidence of vocal fold paralysis in infants undergoing ligation of patent ductus arteriosus. Ann Thor Surg 61:814-6, 1996. 110. Ayyagari R, Smith RJH, Polymeropolous M, Dagner S, Pelias MZ, Wozencraft L, Knpfer MK, Hejtmancik JF. Localization of the Usher syndrome type I gene in the French Acadian population of Louisiana to chromosome 11p14-p15.1 by linkage and haplotype analysis. Ind J Hum Genet 1:93-103, 1996. 111. O’Neill ME, Marietta J, Nishimura D, Wayne S, Van Camp G, Van Laer L, Negrini C, Wilcox ER, Chen A, Fukushima K, Sheffield VC, Smith RJH. A gene for autosomal dominant late-onset progressive non-syndromic hearing loss, DFNA10, maps to chromosome 6. Hum Mole Genet 5:853-856, 1996. 112. Ayyagari R, Nestorowicz A, Li Y, Chandrasekharappa S, Chinault C, Van Tuinen P, Smith RJH, Permult MA, Hejtmancik JF. Construction of a YAC contig encompassing the Usher syndrome type 1C and familial hyperinsulinism loci on chromosome 11p14-15.1. Genome Research 6:504-14, 1996. 113. Wayne S, Der Kaloustian VM, Schloss M, Polomeno R, Scott DA, Sheffield VC, Smith RJH. Localization of the Usher syndrome type ID (Ush1D) to chromosome 10. Hum Mole Genet 10:1689-92, 1996. 114. Smith RJH, Burke DK, Sato Y, Poust RI, Bauman NM. OK-432 therapy for lymphangiomas. Arch Otolaryngol 122:1195-1200, 1996. 115. Reilly J, Thompson J, MacArthur C, Pransky S, Beste D, Smith M, Gray S, Manning S, Walter M, Derkay C, Muntz H, Friedman E, Myer CM, Seibert R, Riding K, Cuyler J, Todd W, Smith RJH. Pediatric aerodigestive foreign body injuries are complications related to timeliness of diagnosis. Laryngoscope 107:17-20, 1997. 116. Bent JP, Smith RJH. Intraoperative diagnosis of primary ciliary dyskinesia. Otolaryngol Head Neck Surg 116:64-67, 1997. 117. Bent JP, Bauman NM, Smith RJH. Endoscopic repair of type IA laryngeal clefts. Laryngoscope 107:282-286, 1997. 118. Marietta J, Mathews K, Burgess R, Moore K, Ni L, Fukushima K, Hejtmancik JF, Smith RJH. Usher syndrome type 1C: Clinical studies and fine mapping the disease locus. Ann Otol Rhinol Laryngol 106:123-128, 1997. 119. Coucke P, Van Camp G, Demirhan O, Kabakkaya Y, Dalemans W, Van Hauwe P, Van Agtmael T, Smith RJH, Parving A, Bolder CHHM, Cremers CWRJ, Willems PJ. The gene for Pendred syndrome is located between D7S501 and D75692 in a 1.7 cM region on chromosome 7q. Genomics 40:48-54, 1997.
  • 14. Smith RJH 14 120. Van Camp G, Willems P, Smith RJH. Nonsyndromic hearing loss: unparalleled heterogeneity. Am J Hum Genet 60:758-64, 1997. 121. Van Camp G, Couck PJ, Kunst D, Schatteman I, Van Velzen D, Marres H, van Ewijk M, Declau F, Van Hauwe P, Meyers J, Kenyon J, Smith SD, Smith RJH, Djelantik B, Cremers CWRJ, Van de Heyning PH, Willems PJ. Linkage analysis of progressive hearing loss in five extended families maps the DFNA2 gene to a 1.25 Mb region on chromosome 1p. Genomics 41:70-74, 1997. 122. Verhoeven K, Van Camp G, Govaerts PJ, Balemans W, Schatteman I, Van Laer L, Smith RJH, Brown MR, Van de Heyning P, Somers T, Offeciers FE, Willems PJ. A gene for autosomal dominant non-syndromic hearing loss (DFNA12) maps to chromosome 11q22-24. Am J Hum Genet 60:1168-73, 1997. 123. Bauman NM, Burke DK, Smith RJH. Treatment of massive or life-threatening hemangiomas with recombinant alpha-2a interferon. Otolaryngol Head Neck Surg 117:99-110, 1997. 124. Carpenter KM, Graham SM, Smith RJH. Facial skeletal growth after endoscopic sinus surgery in the piglet model. Amer J Rinol 11:211-217, 1997. 125. Maschka DA, Bauman NM, McCray PB, Hoffman HT, Karnell MP, Smith RJH. A classification scheme for paradoxical vocal cord motion. Laryngoscope 107:1429-1435, 1997. 126. Smith RJH. Mucoepidermoid carcinoma in a 10-year-old girl. Controversies. Head Neck 19:431-435, 1997. 127. Chen A, Wayne S, Bell A, Ramesh A, Srisailapathy CRS, Scot DA, Sheffield VC, Van Hauwe P, Zbar RIS, Ashley J, Lovett M, Van Camp G, Smith RJH. A new gene for autosomal recessive non-syndromic hearing loss maps to either chromosome 3q or 19p. Am J Med Genet 71:467-471, 1997. 128. Greinwald JH, Scott DA, Marietta JR, Carmi R, Manaligod J, Ramesh A, Zbar RIS, Kraft ML, Elbedour K, Yairi Y, Musy M, Shvorak AB, Srisailapathy CRS, Lovett M, Morton CC, Sheffield VC, Smith RJH. Construction of a PAC and YAC contig encompassing the DFNB7 and DFNB11 region of chromosome 9q13-21. Genome Res 7:879-886, 1997. 129. Brown MR, Tomek MS, Van Lear L, Smith S, Kenyon JB, Van Camp G, Smith RJH. A novel locus for atuosomal dominant non-syndormic hearing loss, DFNA13, maps to chromosome 6p. Am J Hum Genet 61:924-927, 1997. 130. Fischel-Ghodsian N, Bykhovskaya Y, Taylor K, Kahen T, Cantor R, Ehrenman K, Smith RJH, Keithley E. Temporal bone analysis of patients with presbycusis reveals high frequency of mitochondrial mutations. Hearing Res 110:147-154, 1997.
  • 15. Smith RJH 15 131. Smith RJH. Endolymphatic sac obliteration for LVAS. Am J Otol 18:1, 1997. 132. Nester JC, Robinson RA, Smith RJH, Raab SS. Valued based pathology: A cost benefit analysis of the examination of routine and non-routine tonsil and adenoid specimens. Am J Clin Pathol 108:126-126, 1997. 133. Merritt RM, Bent JP, Smith RJH. Suprastomal granulation tissue and pediatric tracheotomy decannulation. Laryngoscope 107:868-871, 1997. 134. Bent JP, Klippert FN, Smith RJH. Management of congenital buccopharyngeal membrane. Cleft Palate-Craniofacial J 34:538-541, 1997. 135. Van Laer L, Van Camp G, van Zuijlen D, Green ED, Verstreken M, Schatteman I, Van de Heyning P, Balemans W, Coucke P, Greinwald JH, Smith RJH, Huizing E, Willems P. Refined mapping of a gene for autosomal dominant progressive sensorineural hearing loss (DFNA5) to a 2-cM region, and exclusion of a candidate gene that is expressed in the cochlea. Eur J Hum Genet 5:397-405, 1997. 136. Van Camp G, Smith RJH. The hereditary hearing loss homepage. J Audiologic Med 6:iii-v, 1997. 137. Scott DA, Sheffield VC, Stone EM, Kraft ML, Smith RJH. Cx26 M34T Variant in a hearing family. Nature 391:32, 1998. 138. Chen AH, Mueller RF, Prasad SD, Greinwald JH, Manaligod J, Muilenburg AC, Van Camp G, Smith RJH. Presymptomatic diagnosis of non-syndromic hearing loss by genotyping. Arch Otolaryngol 124:20-24, 1998. 139. Tomek MS, Brown MR, Mani SR, Ramesh A, Srisailapathy CRS, Coucke P, Zbar RIS, Bell AM, McGuirt WT, Fukushima K, Willems PJ, Van Camp G, Smith RJH. Localization of a gene for otosclerosis to chromosome 15q25-q26. Hum Mole Genet 7:285-290, 1998. 140. Bent JP, Smith RJH. A case report: aerocele after tracheocutaneous fistula closure. Inter J Ped Otorhinolaryngol 42:257-261, 1998. 141. Higgins MJ, Day CD, Smilinich NJ, Ni L, Cooper PR, Nowk NJ, Davies C, de Jong PJ, Hejtmancik JF, Evans GA, Smith RJH, Shows TB. Contig and transcript maps identify candidate genes in the Usher 1C locus. Genome Res 8:57-68, 1998. 142. Smith, RJH. Hemangioma. Controversies. Head Neck 20:69-72,1998. 143. Zbar RIS, Ramesh A, Srisailapathy CRS, Fukushima K, Wayne S, Smith RJH. Passage to India: The search for genes causing autosomal recessive nonsyndromic hearing loss. Otolayrngol Head Neck Surg 118:333-337, 1998. 144. Scott DA, Kraft ML, Carmi R, Ramesh A, Elbedour K, Yairi Y, Srikumari Srisailapathy CR, Rosengren SS, Markham AF, Mueller RF, Lench NJ, Van Camp G, Smith RJH, Sheffield VC. Identification of mutations in the Connexin 26 gene
  • 16. Smith RJH 16 that cause autosomal recessive non-syndromic hearing loss. Hum Mutation 11:387-394, 1998. 145. Connelly SM, Smith RJH. Effects of rigid plate fixation and removal: craniofacial growth in rabbits. Arch Otolaryngol 124:444-447,1998. 146. Lench N, Markham A, Mueller R, Kelsell D, Smith RJH, Willems P, Schatteman I, Capon H, Van de Heyning P, Van Camp G. A Moroccan family with autosomal recessive sensorineural hearing loss caused by a mutation in the gap junction protein gene connexin 26 (GJB2). J Med Genet 35:151-152, 1998. 147. Verhoeven K, Van Laer L, Kirschhofer K, Legan PK, Hughes DC, Schatteman I, Verstreken M, Van Hauwe P, Coucke P, Chen A, Smith RJH, Somers T, Offeciers FE, Van de Heyning P, Richardson GP, Wachtler F, Kimberling WJ, Willems PJ, Govaerts PJ, Van Camp G. Mutations in the human ∀-tectorin gene cause autosomal dominant non-syndromic hearing impairment. Nature Genet 19:60-62, 1998. 148. Van Camp G, Smith RJH. The hereditary hearing loss homepage. Eur Work Group on the Genet of Hear Imp, Info Let 4:1-3, 1998. 149. Smith RJH. Recurrent respiratory papillomatosis. Controversies. Head Neck 20:418-424, 1998. 150. Jain PK, Lalwani AK, Li XC, Singleton TL, Smith TN, Chen A, Deshmukh D, Verma I, Smith RJH, Wilcox ER. A gene for non-syndromic sensorineural deafness (DFNB18) maps to the chromosomal region 11p14-15.1 containing the usher syndrome type 1C gene. Genomics 50:290-292, 1998. 149.Greinwald JH, Wayne S, Chen AH, Zbar RIS, Kraft ML, Prasad S, Ramesh A, Coucke P, Srisailapathy CRS, Lovett M, Van Camp G, Smith RJH. Localization of a novel gene for non-syndromic hearing loss, DFNB17, to chromosome 7q31. Am J Med Genet 78:107-113, 1998. 150.Van Hauwe P, Everett LA, Coucke P, Scott DA, Kraft ML, Ris-Stallpers C, Bolder C, Otten B, de Vijlder JJM, Dietrich NL, Ramesh A, Srisailapathy SCR, Parving A, Cremers CWRJ, Willems PJ, Smith RJH, Green ED, Van Camp G. Two frequent missense mutations in Pendred syndrome. Hum Mole Genet 7:1099-1104, 1998. 151.Bauman NM, Wang D, Jaffe DM, Porter MP, McCulloch TM, Smith RJH, Sandler AD. Role of substance P in the laryngeal chemoreflex. Ann Otol Laryngol Rhinol 107:575-580, 1998. 152.Van Camp G, Willems PJ, Kunst H, Marres H, Smith RJH. Recent developments in genetic hearing impairment. J Audiologic Med 7:120-133, 1998. 153.Mankarious LA, Smith RJH. External rhinoplasty approach for extirpation and immediate reconstruction of congenital midline nasal dermoids: long-term follow-up. Ann Otol Laryngol Rhinol 107:786-790, 1998.
  • 17. Smith RJH 17 154.Scott DA, Greinwald JH, Marietta JR, Drury S, Swiderski RE, Vinas A, DeAngelis MM, Carmi R, Ramesh A, Kraft ML, Elbedour K, Skworak AB, Friedman RA, Srisailapathy CRS, Verhoeven K, Van Camp G, Lovett M, Deininger PL, Batzer MA, Morton CC, Keats BJ, Smith RJH, Sheffield VC. Identification and mutation analysis of a cochlear-expressed, zinc finger protein gene at the DFNB7/11 and dn hearing- loss-loci on human chromosome 9q and mouse chromosome 19. Gene 215:461-469, 1998. 155.Van Laer L, Richardson G, Logan K, van Zuylen D, Verstreken M, Van de Heyning P, McGuirt WT, Smith RJH, Huizing E, Van Camp G. Nonsyndromic hearing impairment DFNA5 is associated with an inactivating mutation in a gene with unknown function. Nature Genet 20:194-197, 1998. 156.Smith RJH, Schwartz C. Branchio-oto-renal syndrome. J Commun Disord 31:411-421, 1998. 157.Bent JP, Manaligod J, Smith RJH. Unilateral laryngeal dysgenesis. Otolaryngol Head Neck Surg 119:712, 1998. 158.Manaligod J, Smith RJH. Familial laryngeal paralysis. Am J Med Genet 77:277-80, 1998. 159.Greinwald JH, Burke DK, Bonthius DJ, Bauman NM, Smith RJH. An update on the treatment of hemangiomas in children with interferon alfa2a. Arch Otolaryngol 125:21-27, 1999. 160.Beaty MM, Wilson JS, Smith RJH. Laryngeal motion during exercise. Laryngoscope 109:136-139, 1999. 161.Green GE, Scott DA, McDonald JM, Woodworth GG, Sheffield VC, Smith RJH. Carrier rates in the Midwestern United States for GJB2 mutations causing inherited deafness. JAMA 281:2211-2216, 1999. 162.Fukushima K, Kasai N, Ueki Y, Nishizaki K, Sugata K, Hirakawa S, Masuda A, Gunduz M, Ninomiya Y, Masuda Y, Sato M, McGuirt WT, Coucke P, Van Camp G, Smith RJH. A gene for fluctuating, progressive autosomal dominant non-syndromic hearing loss, DFNA16, maps to chromosome 2q23-24.3. Am J Human Genet 65:141-50, 1999. 163.Coucke PJ, Van Hauwe P, Kelley PM, Kunst H, Schatteman I, Van Velzen D, Meyers J, Ensink RJ, Verstreken M, Declau F, Marres H, Kastury K, Bhasin S, McGuirt WT, Smith RJH, Cremers CWRJ, Van de Heyning P, Willems PJ, Smith SD, Van Camp G. Mutations in the KCNQ4 gene are responsible for autosomal dominant deafness in four DFNA2 families. Hum Mole Genet 8(7):1321-1328, 1999. 164.Coucke P, Van Hauwe P, Everett LA, Demirhan O, Kabakkaya Y, Dietrich NL, Smith RJH, Coyle E, Reardon W, Trembath R, Willems PJ, Green ED, Van Camp G. Identification of two mutations in the PDS gene in an inbred family with Pendred syndrome. J Med Genet 36:475-477, 1999.
  • 18. Smith RJH 18 165.Van Camp, G, Kunst H, Flothmann K, McGuirt W, Wauters J, Marres H, Verstreken M, Bespalova IN, Burmeister M, Van de Heyning PH, Smith RJH, Willems PJ, Cremers CWRJ, Lesperance MM. A gene for autosomal dominant hearing impairment (DFNA14) maps to a region on chromosome 4p16.3 that does not overlap the DFNA6 locus. J Med Genet 36:532-536, 1999. 166.Friedman RA, Bykhovskaya Y, Sue CM, DiMauro S, Bradley R, Fallis-Cunningham R, Paradies N, Pensak ML, Smith RJ, Groden J, Li XC, Fischel-Ghodsian N. Maternally inherited deafness due to a novel genetic defect. Am J Med Genet 84: 369-72, 1999. 167.Fransen E, Verstreken M, Verhagen WIM, Wuyts P, Huygen PLM, D’Haese P, Robertson NG, Morton C, McGuirt W, Smith RJH, Declau F, Van de Heyning PH, Van Camp G. High prevalence of symptoms of Meniere’s disease in three families with a mutation in the COCH gene. Hum Mole Genet 8(8):1425-29, 1999. 168.Greinwald JH, Burke DK, Sato Y, Poust RI, Kimura K, Bauman NM, Smith RJH. The treatment of lymphangiomas in children: an update of Picibanil (OK-432) sclerotherapy. Otolaryngol Head Neck Surg 121:381-385, 1999. 169.Dutton JM, Goss K, Khubchandani KR, Shah CD, Smith RJH, Snyder JM. Surfactant protein A in rabbit sinus and middle ear. Ann Otol Rhinol Laryngol 108:915-924, 1999. 170.Manaligod JM, Menezes AH, Bauman NM, Smith RJH. Cervical vertebral anomalies in patients with anomalies of the head and neck. Ann Otol Laryngol Rhinol 108:925-933, 1999. 171.Smith RJH, Van Camp G. Non-syndromic hearing impairment: gene linkage and cloning. Int J Pediatr Otorhinolaryngol 49(51):159-164, 1999. 172.Olney DR, Greinwald JH, Smith RJH, Bauman NM. Laryngomalacia and its treatment. Laryngoscope 109:1770-1775, 1999. 173.Jacobs JP, Quintessenza JA, Andrews T, Burke RP, Spektor Z, Delius R, Smith RJH, Elliott MJ, Herberhold C. Tracheal allograft reconstruction: The North American experience and an update on the worldwide pediatric experience. Ann Thorac Surg 68:1043-1051, 1999. 174.McGuirt WT, Prasad SD, Griffith AJ, Kunst HPM, Green GE, Shpargel KB, Runge C, Huybrecths C, Mueller RF, Lynch E, King MC, Brunner HG, Cremers CWRJ, Takanosu M, Li SW, Arita M, Mayne R, Prockop DJ, Van Camp G, Smith RJH. Mutations in COL11A2 cause non-syndromic hearing loss at the DFNA13 locus. Nature Genet 23:413-419, 1999. 175.Bauman NM, Smith RJH. Surgical management of laryngomalacia. Operative Techniques - Otolaryngol - Head Neck Surg 10:253-258, 1999. 176.McGuirt WT, Smith RJH. Connexin 26 as a cause of hereditary hearing loss. Am J Audiol 8:93-100, 1999.
  • 19. Smith RJH 19 177.Smith RJH, Van Camp G. Cloning genes for non-syndromal hearing loss. Br J Audiol 33:271-278, 1999. 178.Sundstrom RA, Van Laer L, Van Camp G, Smith RJH. Autosomal recessive non- syndromic hearing loss. Am J Med Genet 89:123-129, 1999. 179.Van Laer L, McGuirt WT, Yang T, Smith RJH, Van Camp G. Autosomal dominant non-syndromic hearing impairment. Am J Med Genet 89:167-174, 1999. 180.Bauer MF, Rothbauer U, Muhlenbein N, Smith RJH, Gerbitz KD, Neupert W, Brunner M, Hofmann S. The mitochondrial TIM22 preprotein translocase is highly conserved throughout the eukaryotic kingdom. FEBS 464:41-47, 1999. 181.Jun AI, McGuirt WT, Hinojosa R, Green GE, Fischel-Ghodsian N, Smith RJH. Temporal bone histopathology in Connexin 26-related hearing loss. Laryngoscope 110:269-275, 2000. 182.Weston MD, Eudy JD, Fujita T, Yao S-F, Usami S, Cremers C, Greenburg J, Martini A, Moller C, Smith RJ, Sumegi J, Kimberling WJ. Genomic structure and identification of novel mutations in Usherin, the gene responsible for Usher syndrome type IIa (USH2A). Am J Hum Genet 66:1199-1210, 2000. 183.Scott DA, Drury S, Sundstrom RA, Bishop J, Swiderski RE, Carmi R, Ramesh A, Elbedour K, Srisailapathy CRS, Keats BJ, Sheffield VC, Smith RJH. Refining the DFNB7-DFNB11 deafness interval using intragenic polymorphisms in a novel gene, TMEM2. Gene 246:265-274, 2000. 184.Rosenfeld RM, Bhaya MH, Bower CM, Brookhouser PE, Casselbrant ML, Chan KH, Cunningham MJ, Derkay CS, Gray SD, Manning SC, Messner AH, Smith RJH. Impact of tympanostomy tubes on child quality of life. Arch Otolaryngol Head Neck Surg 126:585-593, 2000. 185.Van Camp G, Smith RJH. Maternally inherited hearing impairment. Clin Genet 57:409-414, 2000. 186.Scott DA, Wang R, Kreman TM, Andrews M, McDonald JM, Marietta JR, Smith RJH, Karniski LP, Sheffield VC. Functional differences of the PDS gene product are associated with phenotypic variation in patients with Pendred syndrome and nonsyndromic hearing loss (DFNB4). Hum Mole Genet 9:1709-1715, 2000. 187.Green GE, Smith RJH, Bent JP, Cohn ES. Genetic testing to identify deaf newborns. JAMA 284:1245, 2000. 188.Verhoeven K, Fagerheim T, Prasad S, Wayne S, De Clau F, Balemans W, Verstreken M, Schatteman I, Solem B, Van de Heyning P, Tranebjarg L, Smith RJH, Van Camp G. Refined localization and two additional linked families for the DFNA10 locus for non-syndromic hearing impairment. Hum Genet 107:7-11, 2000.
  • 20. Smith RJH 20 189.Bauman NM, Sandler AD, Bishop WP, Smith RJH. Value of pH probe testing in pediatric patients with extraesophageal manifestations of gastroesophageal reflux disease: a retrospective review. Ann Otol Rhinol Laryngol 109 (Suppl 184):18-24, 2000. 190.Bearer EL, Chen AF, Chen AH, Li Z, Mark H-F, Smith RJH. 2E4/Kaptin - a candidate gene for the hearing loss loci DFNA4. Ann Hum Genet 64:189-196, 2000. 191.Astuto LM, Weston MD, Carney CA, Hoover DM, Cremers CWRJ, Wagenaar M, Moller C, Smith RJH, Pieke-Dahl S, Greenberg J, Ramesar J, Jacobson SG, Ayuso C, Heckenlively JR, Tamayo M, Gorin MB, Reardon W, Kimberling WJ. Genetic heterogeneity of Usher syndrome: analysis of 151 Usher I families. Am J Hum Genet 67:1569-74, 2000. 192.Brunger JW, Murray GS, O'Riordan M, Matthews AL, Smith RJH, Robin NH. Parental attitudes toward genetic testing for pediatric deafness. Am J Hum Genet 67:1621-25, 2000. 193.Manaligod JM, Milam M, Hill SA, Sanders T, Skaggs J, Smith RJH. Age-related mitochondrial DNA mutations in the human larynx. Laryngoscope 110:2123-2128, 2000. 194.Bork JM, Peters LM, Riazuddin S, Bernsteinn SL, Ahmed ZM, Ness SL, Polomeno R, Ramesh A, Schloss M, Srisailpathy CRS, Wayne S, Bellman S, Desmukh D, Ahmed Z, Khan SN, Der Kaloustian VM, CindyLi X, Lalwani A, Riazuddin S, Bitner- Glindzicz M, Nance WE, Liu XZ, Wistow G, Smith RJH, Griffith AJ, Wilcox ER, Friedman TB, Morell RJ. Usher syndrome ID (USH1D) and nonsyndromic recessive deafness DFNB12 are caused by allelic mutations of the novel cadherin-like gene CDH23. Am J Hum Genet 68:28-37, 2001. 195.De Leenheer EMR, Kunst HPM, McGuirt WT, Prasad SD, Brown MR, Huygen PLM, Smith RJH, Cremers CWRJ. Hearing impairment caused by a missense mutation in COL11A2 (DFNA13). Arch Otolaryngol 127:13-17, 2001. 196.Scott KJ, Greinwald JH, Darrow D, Smith RJH. Endobronchial tumors in children: an uncommon clinical entity. Ann Otol Laryngol Rhinol 110:63-69, 2001. 197.Prasad S, Cucci RA, Green GE, Smith RJH. Genetic testing for hereditary hearing loss: Connexin 26 and its allele variants. Hum Mutation 16:502-508, 2001. 198.Van Den Bogaert K, Govaerts PJ, Schatteman I, Brown MR, Caethoven G, Offeciers FE, Somers T, Declau F, Coucke P, Van de Heyning P, Smith RJH, Van Camp G. A second gene for otosclerosis (OTSC2) maps to chromosome 7q34-36. Am J Hum Genet 68:495-500, 2001. 199.Wayne S, Robertson NG, DeClau F, Chen N, Verhoeven K, Prasad S, Tranebjarg L, Morton C, Ryan AF, Van Camp G, Smith RJH. Mutations in the transcriptional activator EYA4 cause late-onset deafness at the DFNA10 locus. Hum Mole Genet
  • 21. Smith RJH 21 10:195-200, 2001. 200.Cucci RA, Prasad S, Kelley PM, Green GE, Storm K, Cohn ES, Van Camp G, Smith RJH. The M34T allele variant of Connexin 26. Genet Testing 4:335-344, 2001. 201.Waldegger S, Moschen I, Ramirez A, Smith RJH, Ayadi H, Lang F, Kubisch C. Cloning and characterization of SLC26A6, a novel member of the solute carrier 26 gene family. Genomics 72:43-50, 2001. 202.Kasai N, Fukushima K, Ueki Y, Prasad S, Nosakowski J, Sugata KI, Sugata A, Nishizaki K, Meyer NC, Smith RJH. Genomic structures of SCN2A and SCN3A - Candidate genes for deafness at the DFNA16 locus. Gene 264:113-22, 2001. 203.Campbell C, Cucci RA, Green GE, Edeal JB, Galer CE, Prasad S, Karniski LP, Sheffield VC, Smith RJH. Pendred syndrome, DFNB4 and PDS - Identification of eight novel mutations and phenotype-genotype correlations. Hum Mut 17:403-11, 2001. 204.Brunger JW, Matthews AL, Smith RJH, Robin NH. Genetic testing and genetic counseling for deafness: The future is here. Laryngoscope 111:715-19, 2001. 205.Shah ZH, Toompuu M, Hakkinen T, Rovio AT, Van Ravenswaay C, De Leenheer EMR, Smith RJH, Cremers FPM, Cremers CWRJ, Jacobs HT. Novel coding-region polymorphisms in mitochondrial seryl-tRNA synthetase (SARSM) and mitoribosomal protein S12 (RPMS12) genes in DFNA4 autosomal dominant deafness families. Hum Mut 17:433-34, 2001. 206.Caldwell GM, Eddy RL, Day CD, Haley LH, Cooper PR, Sait SSJ, Hejtmancik F, Smith RJH, Morton CC, Higgins MJ, Shows TB. Mapping of genes and transcribed sequences in a gene rich 400-kb region on human chromosome 11p15.1∏p14. Cytogenet Cell Genet 92:103-107, 2001. 207.Fransen E, Verstreken M, Bom SJH, de Kok YJM, Wuyts FL, Verhagen WIM, Huygen PLM, Kemperman MH, McGuirt WT, Smith RJH, Van Maldergem LV, Declau F, Lemaire F, Cremers CWRJ, Van de Heyning P, Cremers FPM. A common ancestor for COCH - related cochleovestibular (DFNA9) patients in Belgium and the Netherlands, all bearing the P51S mutation. J Med Genet 38:61-5, 2001. 208.Alagramam KN, Yuan H, Kuehn MH, Murcia CL, Wayne S, Srisailapathy CRS, Lowry RB, Knaus R, Van Laer L, Bernier FP, Schwatrz S, Lee C, Morton CC, Mullins RF, Ramesh A, Van Camp G, Hagemen GS, Woychik RP, Smith RJH. Mutations in the novel protocadherin PCDH15 cause Usher syndrome type 1F. Hum Mole Genet 10:1709-1718, 2001. 209.Robin NH, Dietz C, Arnold JE, Smith RJH. Pediatric otolaryngologists’ knowledge and understanding of genetic testing for deafness. Arch Otolaryngol 127:937-40,
  • 22. Smith RJH 22 2001. 210.Manaligod JM, Skaggs J, Smith RJH. Localization of the gene for familial laryngeal abductor paralysis to chromosome 6q16. Arch Otolaryngol 127: 913-20, 2001. 211.Chen AH, Stephan DA, Hasson T, Fukushima K, Nelissen CM, Chen AF, June AI, Ramesh A, Van Camp G, Smith RJH. MYO1f as a candidate gene for Nonsyndromic deafness, DFNB15. Arch Otolaryngol 127: 921-36, 2001. 212.Smith RJH. Mutation screening for deafness: more than simply another diagnostic test. Arch Otolaryngol 127: 941-42, 2001. 213.De Leenheer EMR, Huygen PLM, Wayne S, Smith RJH, Cremers CWRJ. The DFNA10 phenotype. Ann Otol Rhinol Laryngol 110:861-66, 2001. 214.Liu X-Z, Blanton SH, Bitner-Glindzicz, Pandya A, Landa B, MacArdle B, Rajput K, Sirimanna T, Webb BT, Ping X, Sismanis A, Smith RJH, Nance WE. Further refinement of the Usher 1D locus at 10q21-22. Clinical Genet 60:58-62, 2001. 215.Haberlandt E, Loffler J, Hirst-Stadlmann A, Stockl B, Judmaier W, Fischer H, Heinz- Erian P, Muller T, Utermann G, Smith RJH, Janecke AR. Split-hand/split-foot malformation associated with sensorineural deafness, inner and middle ear malformation, hypotonia, congenital vertical talus, and deletion of eight microsatellite markers in7q21.1-q21.3. J Med Genet 38:405-9, 2001. 216.Van Laer L, Coucke P, Mueller RF, Caethoven G, Flothmann K, Prasad S, Houseman M, Van de Heyning CM, Fransen E, Rowlands J, Cucci RA, Chamberlin GP, Smith RJH, Van Camp G. A common founder for the 35delG connexin 26 (GJB2) gene mutation in non-syndromic hearing impairment. J Med Genet 38:515-8, 2001. 217.Holcomb JD, Jaffe DM, Greinwald JH, Bauman NM, Smith RJH. Non-traumatic atlanto-axial rotary subluxation in the pediatric otolaryngologic patient: A report of four cases. Ann Otol Rhinol Laryngol 110:1137-40, 2001. 218.Hone SW, Smith RJH. Genetics of hearing impairment. Semin Neonatol 6:531-541, 2001. 219.Vescan A, Parnes LS, Cucci RA, Smith RJH, MacNeill C. Cochlear implantation and PDS mutations in monozygotic twins with large vestibular aqueduct syndrome. J Otolaryngol 31:54-7, 2002. 220.Van Den Bogaert K, Govaerts PJ, De Leenheer EM, Schatteman I, Verstreken M, Chen W, Declau F, Cremers CW, Van De Heyning PH, Offeciers FE, Somers T, Smith RJH, Van Camp G. Otosclerosis: a genetically heterogeneous disease involving at least three different genes. Bone 30:624-30, 2002.
  • 23. Smith RJH 23 221.Najmabadi H, Cucci RA, Sahebjam S, Kouchakian N, Farhadi M, Kahrizi K, Arzhangi S, Daneshmandan N, Javan K, Smith RJH. GJB2 mutation in Iranians with autosomal recessive non-syndromic sensorineural hearing loss. Hum Mut 19:572-4, 2002. 222.Green GE, Scott DA, McDonald JM, Teagle HFB, Tomblin BJ, Spencer LJ, Woodworth GG, Knutson JF, Gantz BJ, Sheffield VC, Smith RJH. Performance of Cochlear Implant Recipients with GJB2-related Deafness. Am J Med Genet 109:167-170, 2002. 223.Astuto LM, Kelley PM, Askew JW, Weston MD, Smith RJH, Alswaid AF, Al-Rakaf M, Kimberling WJ. Searching for evidence of DFNB2. Am J Med Genet 109:291-297, 2002. 224.Cryns K, Pfister M, Pennings RJE, Bom SJH, Flothmann K, Caethoven G, Kremer H, Schatteman I, Kolln KA, Toth T, Kupka S, Blin N, Nurnberg P, Thiele H, Van de Heyning PH, Reardon W, Stephens D, Cremers CWRJ, Smith RJH, Van Camp G. Mutations in the WFS1 gene that cause low-frequency sensorineural hearing loss are small non-inactivating mutations. Hum Genet 110:389-394, 2002. 225.Chen W, Campbell CA, Green GE, Van Den Bogaert K, Komodikis C, Manolidis LS, Aconomou E, Kyamides Y, Christodoulou K, Faghel C, Giguère CM, Alford RL, Manolidis S, Van Camp G, Smith RJH. Linkage of Otosclerosis to a Third Locus on Human Chromosome 6p21.3-22.3. J Med Genet 39:473-477, 2002. 226.Bauman NM, Giguere CM, Manaligod JM, Sato Y, Burke DK, Smith RJH. Management of Lymphatic Malformations – If, When and How. Operative Techniques - Otolaryngol - Head Neck Surg 13:85-92, 2002. 227.Smith RJH, Robin NH. Genetic testing for deafness – GJB2 and SLC26A4 as causes of deafness. J Com Dis 35:367-77, 2002. 228.Smith RJH. Deafness: from bench to bedside and back. Lancet 360:656-7, 2002. 229.Giguère CM, Bauman NM, Sato Y, Burke DK, Greinwald JH, Pransky S, Kelley P, Georgeson K, Smith RJH. Treatment of lymphangiomas with Picibanil (OK-432) sclerotherapy – A prospective multi-institutional trial. Arch Otolaryngol 128:1137-1144, 2002. 230.Poetker DM, Sandler AD, Scott DL, Smith RJH, Bauman NM. Survivin expression in juvenile-onset recurrent respiratory papillomatosis. Ann Otol Rhinol Laryngol 111: 957-961, 2002. 231.Naz S, Giguere CM, Kohrman DC, Mitchem KL, Riazuddin S, Morell R, Ramesh A, Srisailpathy S, Deshmukh D, Riazuddin S, Griffith AJ, Friedman TB, Smith RJH, Wilcox ER. Mutations in a novel gene TMIE, are associated with hearing loss at the
  • 24. Smith RJH 24 DFNB6 locus. Am J Hum Genet 71:632-6, 2002. 232.Vervoort V, Smith RJH, O’Brien J, Schroer R, Abbott A, Stevenson RE, Schwartz CE. Genomic rearrangements of EYA1 account for a large fraction of families with BOR syndrome. European J Hum Genet 10:757-66, 2002. 233.Giguère CM, Bauman NM, Smith RJH. New Treatment Options for Lymphangioma in Infants and Children. Ann Otol Rhinol Laryngol 111:1066-1075, 2002. 234.Cryns K, Thys S, Van Laer L, Oka Y, Pfister M, Van Nassauw L, Smith RJH, Timmermans J-P, Van Camp G. The WFS1 gene, responsible for low frequency sensorineural hearing loss and Wolfram syndrome, is expressed in a variety of inner ear cells. Histochem Cell Bio 119:247-256, 2003. 235.Smith RJH, Huygen PLM. Making sense of nonsyndromic deafness. Arch Otolaryngol 129:405-410, 2003. 236.Hone SW, Smith RJH. Genetic screening for hearing loss. Clinic Otolaryngol 28:285-90, 2003. 237.Liu XL, Ouyang XM, Xia XJ, Zheng J, Pandya A, Li F, Du LL, Welch KO, Petit C, Smith RJH, Webb BT, Yan D, Arnos KS, Corey D, Dallos P, Balkany T, Nance WE, Chen ZY Prestin, a cochlear motor protein, is defective in non-syndromic hearing loss. Hum Mole Genet 12:1155-62, 2003. 238.Alford RL, Friedman TB, Keats BJB, Kimberling WJ, Proud VK, Smith RJH, Arnos KS, Korf BR, Rehm HL, Toriello HV. Early childhood hearing loss: clinical and molecular genetics. An educational slide set of the American College of Medical Genetics. Genet Med 5:338-341, 2003. 239.Van Laer L, Cryns K, Smith RJH, Van Camp G. Nonsyndromic hearing loss. Ear Hear 24:275-288, 2003. 240.Chang EH, Van Camp G, Smith RJH. The role of connexins in human disease. Ear Hear 24:314-323, 2003. 241.Van den Bogaert K, Smith RJH, Govaerts PJ, Van Camp G. Otosclerosis. Audiolog Med 1:33-36, 2003. 242.Green GE, Mueller RF, Cohn ES, Avraham KB, Kanaan M, Smith RJH. Audiological manifestations and features of Connexin 26 deafness. Audiolog Med 1:5-11, 2003. 243.Kolln KA, Smith RJH. Pendred syndrome. Audiolog Med 1:71-76, 2003.
  • 25. Smith RJH 25 244.Robin NH, Smith RJH, Matthews AL. Genetic testing for deafness in clinical practice. Audiolog Med 1:89-93, 2003. 245.Cryns K, Sivakumaran TA, Van den Ouweland JMW, Pennings RJE, Cremers CWRJ, Flothmann K, Young TL, Smith RJH, Lesperance MM, Van Camp G. Mutational spectrum of the WFS1 gene in Wolfram syndrome, nonsyndromic hearing impairment, diabetes mellitus and psychiatric disease. Hum Mut 22:275-87, 2003. 246.Zhu M, Yang T, Wei S, DeWan AT, Morell RJ, Elfenbein JL, Fisher RA, Leal SM, Smith RJH, Friderici KH. Mutations in the gamma actin gene (ACTG1) cause dominant progressive deafness (DFNA20/26). Am J Hum Genet 73:1082-91, 2003. 247.Bayaz ı t YA, Cable BB, Cataloluk O, Kara C, Chamberlin P, Smith RJH, Kanl ı kama M, Ozer E, Cakmak EA, Mumbuc S, Arslan A. GJB2 gene mutations causing Familial hereditary deafness in Turkey. Int J Ped Otolaryngol 67:1331-37, 2003. 248.Bauer PW, Geers AE, Brenner C, Moog JS, Smith RJH. The Effect of GJB2 Allele Variants on Performance After Cochlear Implantation. Laryngoscope 113:2135-41, 2003. 249.del Castillo I, Moreno-Pelayo MA, del Castillo FJ, Brownstein Z, Marlin S, Adina Q, Cockburn DJ, Pandya A, Siemering KR, Chamberlin GP, Ballana E, Wuyts W, Maciel-Guerra AT, Álvarez A, Villamar M, Shohat M, Abeliovich D, Dahl HM, Estivill X, Gasparini P, Hutchin T, Nance WE, Sartorato EL, Smith RJH, Van Camp G, Abraham KB, Petit C, Moreno F. Prevalence and Evolutionary Origins of the del(GJB6-D13S1830) Mutation in the DFNB1 Locus in Hearing Impaired Subjects: a Multicenter Study. Am J Hum Genet 73: 1452-58, 2003. 250.Prasad S, Kölln KA, Cucci RA, Trembath RC, Van Camp G, Smith RJH. Pendred syndrome and DFNB4 - Mutation screening of SLC26A4 by denaturing high-performance liquid chromatography and the identification of seven novel mutations. Am J Med Genet 124A:1-9, 2004. 251.Mhatre AN, Li J, Chen AF, Yost CS, Smith RJH, Kindler CH, Lalwani AK. Genomic Structure, Cochlear Expression and Mutation Screening of TWIK-2, a Candidate Gene for DFNA4. J Neuroscience Res 75:25-31, 2004. 252.Snoeckx RL, Ensink RJH, Kremer H, Flothmann K, Smith RJH, Cremers CWRJ, Van Camp G. A novel locus for autosomal dominant nonsyndromic hearing loss, DFNA31, maps to chromosome 6p21.3. J Med Genet 41:11-13, 2004. 253.Cryns K, Orzan E, Murgia A, Huygen PLM, Moreno F, del Castillo I, Chamberlin GP, Azaiez H, Prasad S, Cucci RA, Leonardi E, Govaerts PJ, Van de Heyning PH, Van de Heyning CM, Smith RJH, Van Camp G. A genotype-phenotype correlation
  • 26. Smith RJH 26 for GJB2 (connexin 26) deafness. J Med Genet (In press). 254.Smith RJH. The Clinical Application of Genetic Testing for Deafness. Am J Med Genet (In press). 255.Chang EH, Menezes M, Meyer NC, Cucci RA, Vervoort VS, Schwartz CE, Smith RJ Branchio-Oto-Renal Syndrome - The Mutation Spectrum in EYA1 and Its Phenotypic Consequences. Hum Mut (In press). 256.Hildebrand MS, de Silva MG, Klockars T, Rose E, Price M, Smith RJH, McGuirt WJ, Christopoulos H, Petit C, Dahl HM. Characterization of DRASIC in the mouse inner ear. Hearing Res (In Press). 257.Cable BB, Manaligod JM, Bauman NM, Smith RJH. Pediatric airway reconstruction - principles, decision-making and outcomes at the University of Iowa Hospitals and Clinics. Ann Otol Rhinol Laryngol (In press). 258.Michaud A-P, Bauman NM, Burke DK, Manaligod JM, Smith RJH. Spastic diplegia and other motor disturbances in infants receiving interferon-alpha. Laryngoscope (In press). Chapters 1. Smith RJH. Hearing Impairment. In: Health & Medical Horizons. New York: MacMillan, 1985:108-121. 2. Smith RJH. The Medical Diagnosis and Treatment of Hearing Loss in Children. In: Cummings CW, Fredrickson JM, Harker LA, Krause CJ, Schuller DE, eds: Otolaryngol Head Neck Surg. St. Louis, Toronto: CV Mosby, 1986: 3225-3247. 3. Smith RJH. Non-Neoplastic Diseases of the Salivary Glands. In: English GM, eds. Otolaryngology, 1990. 4. Smith RJH. Laryngeal Stenosis; Laryngeal Trauma; Laryngeal Papilloma; Laryngeal Hemangioma; Laryngeal Neoplasm. In: Oski FA, DeAngelis C, Feigin RD, Warshaw JB, eds. Principles and Practice of Pediatrics. Philadelphia: JB Lippincott, 1989:1350-59. 5. Smith RJH, Katz CD. Neuroblastoma of the Head and Neck. In: Pochedly C, ed: Neuroblastoma: Tumor Biology & Treatment. RC Press 1990: 245-256. 6. Frankenthaler R, Goepfert H, Smith RJH. Thyroid Cancer in Children. In: Falk SA, ed: Thyroid Disease: Endocrine, Surgery, Nuclear Medicine Radiotherapy. New York: Rave Press, 1990:553-563. 7. Ayyagari R, Smith RJH, Lee EC, Kimberling WJ, Jay M, Bird A, Hejtmancik JF. Heterogeneity of Usher Syndrome Type I. In: Anderson RE, Hollyfield JG, LaVail MM, Orzalesi N, eds: Applications. Plenum Pub Corp, 1993:127-133.
  • 27. Smith RJH 27 8. Bauman NM, Smith RJH. Laryngeal Stenosis; Laryngeal Trauma; Laryngeal Papilloma; Laryngeal Hemangioma; Laryngeal Neoplasm. In: Ski FA, DeAngelis C, Feigin RD, Warshaw JB, eds: Principles and Practice of Pediatrics. Philadelphia: JB Lippincott Co, 1994: 1477-1487. 9. Bauman NM, Smith RJH. Diseases of the Labyrinthine Capsule. In: Bluestone C, Stool S, Kenna M; eds: Pediatric Otolaryngology. W.B. Saunders Co, 1995:677-687. 10. Angeli S, Smith RJH. Hereditary Hearing Impairment. In: Ballenger JJ, Snow J, Jr.; eds: Diseases of the Nose, Throat, Ear, Head and Neck 15th edition. Williams & Wilkins, 1996:1075-1087. 11. Bauman N, Smith RJH. Xeroderma Pigmentosa and Basal Cell Nevus Syndrome. In: Weber R, Miller M, Goepfert H; eds: Basal and Squamous Cell Skin Cancers of the Head and Neck. Williams & Wilkins, Baltimore, 1995:37-47. 12. Smith RJH. Laryngotracheoplasty (Chpt. 223). In: Bailey B, Calhoun K, Coffey A, Neely JG, eds: Atlas of Head and Neck Surgery. Lippincott-Raven Publishers, Philadelphia, 1996:584-585. 13. Smith RJH. Posterior Cricoid Grafting (Chpt. 224). In: Bailey B, Calhoun K, Coffey A, Neely JG, eds: Atlas of Head and Neck Surgery. Lippincott-Raven Publishers, Philadelphia, 1996:586-590. 14. Smith RJH. Choanal Atresia Repair-Transnasal (Chpt. 276). In: Bailey B, Calhoun K, Coffey A, Neely JG, eds: Atlas of Head and Neck Surgery. Lippincott-Raven Publishers, Philadelphia, 1996:714-715. 15. Smith RJH. Choanal Atresia Repair-Transpalatal (Chpt. 277). In: Bailey B, Calhoun K, Coffey A, Neely JG, eds: Atlas of Head and Neck Surgery. Lippincott- Raven Publishers, Philadelphia, 1996:716-720. 16. Smith RJH, Kimberling WJ. Branchio-Oto-Renal Syndrome (Chpt. 19). In: Martini A, Read A, Stephens D, eds: Genetics and Hearing Impairment. Whurr Publishers, London, England, 1996:180-185. 17. Kimberling WJ, Smith RJH. Usher Syndrome (Chpt. 14). In: Martini A, Read A, Stephens D, eds: Genetics and Hearing Impairment. Whurr Publishers, London, England, 1996:141-146. 18. Bauman NM, Smith RJH. Recurrent Respiratory Papillomatosis. Pediatr Clin N America 43(6):1385-1402, 1996. 19. Ayyagari R, Li A, Nestorowicz A, Li Y, Smith RJH, Permutt MA, Hejtmancik JF. Usher Syndrome Type 1C: Localization to chromosome 11p14 and construction of a YAC contig. In: La Vail: Degenerative Retinal Diseases. Plenum Press, New York, 1997: 303-312.
  • 28. Smith RJH 28 20. Smith RJH, Ramesh A, Srisailapathy CRS, Fukushima K, Wayne S, Chen A, Van Laer L, Ashley J, Zbar RIS, Lovett M, Van Camp G. Homozygosity mapping applied to hereditary hearing impairment - localizing recessive deafness genes (Chpt. 13). In: Stephens D, Read A, Martini A (eds): Developments in Genetic Hearing Impairment. Whurr Publishers, 1998:77-90. 21. Smith RJH, Scott DA, Sheffield VC, Van Camp G. DFNB1, GeneClinics ( 1998-2000. 22. Smith RJH, Scott DA, Sheffield VC, Van Camp G. DFNA3, GeneClinics ( 1998-2000. 23. Smith RJH, Everett LA, Green ED, Scott DA, Sheffield VC, Van Camp G, Van Hauwe P. Pendred syndrome. GeneClinics 1998-2000. 24. Green G, Smith RJH. Hereditary Hearing Loss and Deafness. GeneClinics ( 1998-2000. 25. Green G, Prasad SD, Smith RJH. Branchio-Oto-Renal Syndrome. GeneClinics ( 1998-2000. 26. Greinwald JH, Smith RJH. Hereditary Hearing Impairment. In Gates G, ed: Current Therapy in Otolaryngology - Head & Neck Surgery. St. Louis: Mosby-Year Book, Inc., 1998:34-40. 27. Bent JP, Smith RJH. The Cleft Palate Ear. In: Grundfast K, Lalwani A, eds: Pediatric Otology/Neurotology. Philadelphia: J.B. Lippincott, 1998:627-634. 28. Smith RJH, Robinson RA. Head and Neck Malignancies in Children. In: Cummings CW, Fredrickson JM, Harker LA, Krause CJ, Schuller DE, Richardson M, eds: Otolaryngology - Head and Neck Surgery. St. Louis, Toronto: CV Mosby, 1998:(5) 229-247. 29. Smith RJH. Nasopharyngeal Stenosis and Velopharyngeal Insufficiency. ABO Recertification Study Guide American Academy of Otolaryngology–Head and Neck Surgery Foundation, 1998. 30. Manaligod JM, Smith RJH. The Syndromal Child. In: Bailey BJ. Head & Neck Surgery - Otolaryngology. Lippincott-Raven, 1998:1259-1274. 31. Smith RJH, Van Camp G. Non-syndromic hearing impairment - gene linkage and cloning. In: Ruben RJ, Karma P, eds: Advances in Pediatric otorhinolaryngology CD-ROM. Elsevier Science, 1999. 32. Smith RJH, Greinwald JH. Laryngeal Stenosis; Laryngeal Trauma; Laryngeal Papilloma; Laryngeal Hemangioma; Laryngeal Neoplasm. In: Ski FA, DeAngelis C, Feigin RD, Warshaw JB, eds: Principles and Practice of Pediatrics. Philadelphia: JB Lippincott Co, 1999:1230-1240.
  • 29. Smith RJH 29 33. Green GE, Bauman NM, Smith RJH. Pathogenesis and Treatment of Juvenile- Onset Recurrent Respiratory Papillomatosis. Otolaryngol Clin N America, 33(1):187-207, 2000. 34. Bent JP, Hebert RL, Smith RJH. Pediatric Neck Neoplasms. In: Wetmore R, Muntz, McGill T, eds: Pediatric Otolaryngology: Principles and Practice Pathways. Thieme Publishers, New York, 2000:993-1019. 35. Hegarty JL, Smith RJH. Tinnitus in Children. In: Tyler R. Tinnitus Handbook. Singular Publishing, 2000:243-262. 36. McGuirt WT, Lesperance MM, Wilcox ER, Chen AH, Van Camp G, Smith RJH. Characterization of Autosomal Dominant Non-Syndromic Hearing Loss Loci: DFNA 4, 6, 10 and 13. In: Kitamura K, Steel KP, eds: Advances in Oto-Rhino- Laryngology. S. Karger Ag, Basel, 2000:84-96. 37. Van Camp G, Coucke PJ, Van Hauwe P, Van Laer L, Verhoeven K, Wuyts F, Smith RJH. DFNA 2, 4, 8, 12. In: Kitamura K, Steel KP, eds: Advances in Oto- Rhino-Laryngology. S.Karger Ag, Basel, 2000:68-77. 38. Fukushima K, Ueki Y, Smith RJH. Homozygosity Mapping to Localize Genes Causing Autosomal Recessive Non-Syndromic Hearing Loss. In: Kitamura K, Steel KP, eds: Advances in Oto-Rhino-Laryngology. S.Karger Ag, Basel, 2000:152-157. 39. Chen AH, Fukushima K, McGuirt WT, Smith RJH. DFNB15; Autosomal Recessive Non-Syndromic Hearing Loss Gene – Chromosome 3q, 19p or Digenic Recessive Inheritance. In: Kitamura K, Steel KP, eds: Advances in Oto-Rhino-Laryngology. S.Karger Ag, Basel, 2000:171-175. 40. Nichols BE, Smith RJH. Usher Syndrome. In: Weingeist T, Gold D, eds: The Eye in Systemic Disease. Lippincott Williams & Wilkins, 2000. 41. Greinwald JH, Smith RJH. Langerhans’ Cell Histiocytosis. In: Jackler R, Driscoll CLW, eds: Tumors of the Ear and Temporal Bone. Lippincott Williams & Wilkins, 2000:417-431. 42. Smith RJH, Van Camp G. Nonsyndromic Hereditary Hearing Loss and Deafness, DFNB1 (GJB2). GeneClinics ( 2001. 43. Smith RJH, Van Camp G. Nonsyndromic Hereditary Hearing Loss and Deafness, DFNA3 (GJB2). GeneClinics ( 2001. 44. Smith RJH, Van Camp G. Pendred syndrome. GeneClinics 2001. 45. Smith RJH, Green GE, Van Camp G. Hereditary Hearing Loss and Deafness. GeneClinics ( 2001. 46. Green GE, Smith RJH. Delineation of genetic components of communication disorders. In: Gerber SE, Ed: Handbook of Genetic Communicative Disorders. Academic Press, 2001: 12-30.
  • 30. Smith RJH 30 47. Smith RJH, Manaligod JM. The syndromal Child. In: Bailey BJ, ED: Head & Neck Surgery - Otolaryngology. Lippincott Williams & Wilkins, Philadelphia, 2001: 1039-51. 48. Smith RJH. Anterior and Posterior Laryngotracheal Reconstruction. In: Bailey B, Calhoun K, Coffey A, Neely JG, eds: Atlas of Head and Neck Surgery, 2nd Edition. Lippincott Williams & Wilkins, Philadelphia, 2001: 622-26. 49. Smith RJH. Posterior Glottic Split with Cartilage Graft. In: Bailey B, Calhoun K, Coffey A, Neely JG, eds: Atlas of Head and Neck Surgery, 2nd Edition. Lippincott Williams & Wilkins, Philadelphia, 2001: 620-22. 50. Smith RJH. Transnasal Repair of Choanal Atresia. In: Bailey B, Calhoun K, Coffey A, Neely JG, eds: Atlas of Head and Neck Surgery, 2nd Edition. Lippincott Williams & Wilkins, Philadelphia, 2001: 756-8. 51. Smith RJH. Transpalatal Repair of Choanal Atresia. In: Bailey B, Calhoun K, Coffey A, Neely JG, eds: Atlas of Head and Neck Surgery, 2nd Edition. Lippincott Williams & Wilkins, Philadelphia, 2001: 758-62. 52. Green GE, Smith RJH. Congenital Hearing Loss. In: Alper, Myers, Eibling, Eds: Decision Making in Ear, Nose, and Throat Disorders. Harcourt Health Sciences. W.B. Saunders, Philadelphia. 2001: 8-12. 53. Tyler RS, Smith RJH. Management of Tinnitus in Children. In: Newton V, ed: Paediatric Audiological Medicine. Whurr Publishers LTD. 2002: 405-422. 54. Smith RJH, Hone SW. Hereditary Hearing Impairment. In: Ballenger JJ, Snow J, Jr.; eds: Diseases of the Nose, Throat, Ear, Head and Neck 16th edition. Williams & Wilkins. 2002: 324-344. 55. Hone SW, Smith RJH. Understanding inner ear physiology at the molecular level. In: Cremers CWRJ, Smith RJH, eds. Genetic Hearing Impairment – Its Clinical Presentations. Basel, Karger, 2002:1-11. 56. De Leenheer EMR, Huygen PLM, Wayne S, Verstreken M, Declau F, Van Camp G, Van de Heyning PH, Smith RJH, Cremers CWRJ. DFNA10/EYA4 – the clinical picture. In: Cremers CWRJ, Smith RJH, eds: Genetic Hearing Impairment. Adv Otorhinolarygngol. Basel, Karger, 2002:73-78. 57. De Leenheer EMR, McGuirt WT, Kunst HPM, Huygen PLM, Smith RJH, Cremers CWRJ. The phenotype of DFNA13/COL11A2. In: Cremers CWRJ, Smith RJH, eds: Genetic Hearing Impairment. Adv Otorhinolarygngol. Basel, Karger, 2002:85-91. 58. McGuirt WT, Prasad SD, Cucci RA, Green GE, Smith RJH. Clinical Presentation of DFNB1. In: Cremers CWRJ, Smith RJH, eds: Genetic Hearing Impairment. Adv Otorhinolarygngol. Basel, Karger, 2002:113-119.
  • 31. Smith RJH 31 59. Hone SW, Smith RJH. Medical Evaluation of Pediatric Hearing Loss: Laboratory, Radiographic and Genetic Testing. Otolaryngol Clin N America, 2002:35(4):751-765. 60. Smith RJH, Giguère C, Bauman NM. Diseases of the Labyrinthine Capsule. In: Bluestone C, Stool S, Alper C, Arjmand E, Casselbrandt M, Dohar J, Yellon R, eds: Pediatric Otolaryngology. W.B. Saunders Co., 2003:817-828. 61. Green GE, Bauman NM, Smith RJH. Juvenile-onset recurrent respiratory papillomatosis. In: Ossoff R, Shapshay S, Woodson G, Netterville JL, eds: The Larynx. Lippincott Williams & Wilkins, 2003. 62. Smith RJH, Hone SW. Genetic Screening for Deafness. Pediatr Clin N America, 2003: 50(2):315-329. 63. McGuirt WT, Van Camp G, Smith RJH. COL11A2 and DFNA13. In: Willems P; ed: The Genetics of Hearing Loss (In press). 64. Wayne S, De Leenheer E, Cremers CWR, Smith RJH. DFNA10 and Eyes Absent 4. In: Willems P; ed: The Genetics of Hearing Loss (In press). Pamphlets 1. Smith RJH. Academy Pamphlet: Second-Hand Smoke. ASPO and AAO-HNS, 1992. Books/Manuals 1. Tribby R, Fagan C, Conrad C, Leick N, Shalla A, Smith RJH. Tracheostomy Care for Infants and Children. University of Iowa Hospitals and Clinics, 1992. 2. Cremers CWRJ, Smith RJH eds. Genetic Hearing Impairment – Its Clinical Presentations. Karger Publishers, Basel, Switzerland, 2002. CDs 1. Bent JP, Smith RJH. Endoscopic sinus surgery. University of Iowa, 1995. 2. Alford RL, Friedman TB, Keats BJB, Kimberling WJ, Proud VK, Smith RJH, Arnos KS, Korf BR, Rehm HL, Toriello HV. Early childhood hearing loss: clinical and molecular genetics. An educational slide set of the American College of Medical Genetics, 2003. B. Areas of Research Interest/Current Projects Clinical Research Interests Functional matrix theory
  • 32. Smith RJH 32 1. Functional endoscopic sinus surgery and facial growth 2. Microplating and facial growth Laryngology 1. Esophageal - laryngeal reflex development 2. Interarytenoid notch height and aspiration 3. Recurrent respiratory papillomatosis Vascular Anomalies 1. Lymphangiomas 2. Hemangiomas Basic Science Research Interests Hereditary deafness 1. Branchio-oto-renal syndrome 2. Usher syndrome 3. Pendred syndrome 4. Deaf mouse mutants 5. Molecular genetic studies of archival temporal bones 6. Non-syndromic hearing loss C. Grants Received Federal 1. (a) NIH: Molecular Genetics, DNA Linkage Study of Usher Syndrome; USPHS Grant No. RR-05425; PI : RJH Smith; (b) 40% effort, no salary; (c) 7/1/87-6/30/88; Direct costs: $10,000 over 1 year 2. (a) NIDCD: Auditory Processing in Hearing Impaired Children; R29DC00421; PI: Susan Jerger; (b) 5% effort, no salary; (c) 4/1/89-3/31/94; (d) Direct costs: $60,000 per year 3. (a) NIDCD: A collaborative study of Usher syndrome type I; R01 DC02046; PI: RJH Smith; (b) 20% salary; (c) 7/1/93-6/30/97; (d) Direct costs: $476,673 4. (a) NIDCD: Non-Syndromic Hearing Loss - A Collaborative Study; R01 DC02842; PI: RJH Smith; (b) 30% effort, 30% salary; (c) 7/1/96-6/30/00; (d) Direct costs: $1,076,674 5. (a) Institutional National Research Service Award (T32): Research Training Program for Otolaryngology; Co-Director: RJH Smith; (b) 7/1/98-6/30/03; (c) Direct costs: $662,025 6. (a) NIDCD: Statement of Work: Support for Hereditary Hearing Loss Homepage - RJH Smith; (b) No salary; (c) 3 years, 1998-2000; (d) $3400.00/year.
  • 33. Smith RJH 33 7. (a) NIDCD: Autosomal Dominant Non-Syndromic Hearing Loss; R01 DC03544; PI: RJH Smith; (b) 30% effort, 30% salary; (c) 7/1/98-6/30/02; (d) Direct costs: $923,261 8. (a) NIDCD: Non-Syndromic Hearing Loss - A Collaborative Study; RO1 DC02842 (competitive renewal); PI: RJH Smith; (b) 30% effort, 30% salary; (c) 7/1/00 - 6/30/04; (d) Direct costs: $1,284,216 9. (a) FD-R-001774-01: OK-432 Sclerotherapy, A Multicenter Trial; PI: RJH Smith; (b) No salary; (c) 10/1/00-9/30/04; (d) Total costs: $727,982 10. (a) NIDCD: Autosomal Dominant Non-Syndromic Hearing Loss; Administrative Supplement for Targeted Mouse Mutants; R01 DC03544; PI: RJH Smith; (b) 8/01/01-7/31/02; (c) Direct costs: $33,745 11. (a) NIDCD: Otosclerosis - A Molecular Genetic Study; R01DC05218; PI: RJH Smith; (b) 20% effort, 20% salary; (c) 5/1/02-4/30/07; (d) Direct costs: $1,250,000 12. (a) NIDCD: Autosomal Dominant Non-Syndromic Hearing Loss; R01 DC03544; PI: RJH Smith; (b) 20% effort, 20% salary; (c) 7/1/02-6/30/07; (d) Direct costs: $1,687,824 College of Medicine 1. (a) Carver Trust: Headbobber Transgenic Mouse Mutant; PI: RJH Smith; (b) 5% effort, no salary; (c) 1/1/92-12/31/93; (d) Direct costs: $7,500 2. (a) Educational Development Grant: Development of a CD-ROM Instructional Course on Pediatric Sinusitis; PI: JP Bent; RJH Smith; (b) 5% effort, no salary; (c) 1/1/95-12/31/95; (d) Direct costs: $5,000 3. (a) Process Improvement Grant Proposal: Myringotomy +/- Adenoidectomy; PI: RJH Smith; (b) 1% effort, no salary; (c) 7/1/95-12/31/95; (d) Direct costs: $6,240 Other 1. (a) Deafness Research Foundation: Usher Syndrome - A DNA Linkage Study; PI: RJH Smith; (b) 40% effort, no salary; (c) 1/01/88-12/31/90; (d) Direct costs: $45,000 over 3 years 2. (a) Hoffmann-La Roche: Therapy of Massive Capillary Hemangioma and Lymphangioma with Recombinant Human Interferon Alpha-2A; PI: RJH Smith; (b) 5% effort, no salary; (c) 1/1/91-6/30/93; (d) Direct costs: $12,500 over 18 months 3. (a) The National Retinitis Pigmentosa Foundation, Inc.: A Molecular Genetic Study of Usher Syndrome; PI: RJH Smith; (b) 40% effort, no salary; (c) 9/1/90-8/31/93; (d) Direct costs: $223,077 over 3 years
  • 34. Smith RJH 34 4. (a) Children's Miracle Network Telethon: Clinical Applications of Otoacoustic Emissions; PI: RJH Smith; (b) 5% effort, no salary; (c) 1/1/92-12/31/92; (d) Direct costs: $7,500 5. (a) The National Retinitis Pigmentosa Foundation, Inc.: A Molecular Genetic Study of Usher Syndrome; PI: RJH Smith; (b) 20% effort, no salary; (c) 9/1/93- 8/31/94; (d) Direct costs: $30,000 6. (a) Children’s Miracle Network Telethon: Esophago-Laryngeal Adductor Reflex; PI: NM Bauman; Co PI: RJH Smith; (b) No salary (c) 1/1/95 - 12/31/95; (d) Direct costs: $7,200 7. (a) Daiichi Pharmaceutical Corporation: Ofloxacin Otic Solution in the Treatment of Purulent Otorrhea in Adolescent and Adult Patients with Chronic Perforation of Tympanic Membranes; PI: RJH Smith; (b) 5% effort, no salary; (c) 5/5/95-11/5/95; (d) Direct costs: $21,600 8. (a) Daiichi Pharmaceutical Corporation: A Multicenter, Prospective with Historical and Current Practice Control, Open-Label Study to Examine the Safety and Efficacy of Ofloxacin Otic Solution in the Treatment of Acute Purulent Otorrhea; PI: RJH Smith; (b) 5% effort, no salary; (c) 5/5/95-11/5/95; (d) Direct costs: $18,000 9. (a) Obermann Center for Advanced Studies Spelman Rockefeller (CASSPR) grant: HLA Typing in Pediatric Patients with Recurrent Respiratory Papillomatosis; PIs: NM Bauman, RJH Smith; (b) No salary (c) 7/1/96-6/30/97; (d) Direct costs: $5,000 10. (a) Children’s Miracle Network Telethon: Pediatric Laryngoscopes; PIs: NM Bauman, RJH Smith (b) No salary (c) 1997 (d) Equipment costs: $7,500 11. (a) American Otological Society: Otosclerosis - A Molecular Genetic Study; PI: RJH Smith; (b) No salary (c) 7/1/98-6/30/99; (d) Direct costs: $40,000 12. (a) Chugai Pharmaceutical Corporation: OK-432 in the Treatment of Lymphangiomas; PI: RJH Smith; (b) No salary; (c) 4/1/99-3/31/04; (d) Direct costs: $150,000 13. (a) US PHS-CDC: Establishment of National Registry for Pediatric Patients with Recurrent Respiratory Papillomatosis Disease; PI: RJH Smith; (b) No salary; (c) 7/17/00-6/30/01; (d) $1,380. 14. (a) Stressgen Biotechnologies: Pediatric Recurrent Respiratory Papillomatosis (RRP) Clinical Trial, SGN-00101-0005; Co-I: RJH Smith; (b) No salary; (c) 7/01-6/03; (d) $35,000. D. Grants Mentored
  • 35. Smith RJH 35 1. Jose Manaligod, MD – KO8 2. John Greinwald, MD – KO8 III. SERVICE A. Professional Activities National 1986-88 Task Force on New Materials: American Academy Otolaryngology-Head and Neck Surgery and the American Board of Otolaryngology 1989-95 Home Study Course: American Academy of Otolaryngology - Head and Neck Surgery and the American Board of Otolaryngology (Chairman, 1994-95) 1992-94 Program Chair: American Society of Pediatric Otolaryngology 1994-98 Secretary: American Society of Pediatric Otolaryngology 1998-99 President-Elect: American Society of Pediatric Otolaryngology 1999-00 President: American Society of Pediatric Otolaryngology Local 1986-90 Texas Children's Hospital Surgery Preparation Task Force 1987-90 Deputy Chief, Department of Otolaryngology, Harris County Hospital District Medical Staff 1988.90 Houston School for the Deaf Medical Advisory Board Editorial Responsibilities Editor/Associate Editor 1984-1995 Abstract Editor (Pediatric Otolaryngology), Head and Neck 1990-2001 Editor, Controversies, Head and Neck 2001-2003 Associate Editor, Annals of Otology, Laryngology, & Rhinology 2003- Associate Editor, Gene Tests, Gene Clinics 2004- Editor, Annals of Otology, Laryngology, & Rhinology Editorial Boards 1990- Editorial Board, Head and Neck 1990- Editorial Board, Otolaryngology-Head and Neck Surgery 1997- Editorial Board, The American Journal of Otology 1997- Editorial Board, Annals of Otology, Laryngology, & Rhinology 1997- Editorial Board, Archives of Otolaryngology-Head and Neck Surgery 1998 1998- Editorial Board, International Journal of Pediatric Otorhinolaryngology 1999- Editorial Board, Acta Oto-Laryngologica 2003- Editorial Board, Audiological Medicine 2003- Editorial Board, American Journal of Medical Genetics Journal Peer Review Board 1984- Head and Neck 1987- Otolaryngology-Head and Neck Surgery
  • 36. Smith RJH 36 1988 Neurofibromatosis 1989- American Journal of Medical Genetics 1991- Ear and Hearing 1991- Annals of Otology, Rhinology and Laryngology 1992- Genomics 1992- Archives of Otolaryngology 1994- Human Molecular Genetics 1994- American Journal of Human Genetics 1996- Nature Genetics 1996- The Journal of Pediatrics 1996- Clinical Genetics 1996- European Journal of Human Genetics 1997- Journal of the American Medical Association 1998- Nature 1999 1998- Lancet 2000 1998- Genome Research 2000- Human Mutation 2001- New England Journal of Medicine Collegiate, University and National Committees University 1991- Medical Records Subcommittee 1993- ACS Committee 1993- Clinic Operations Committee 1996- Children’s Hospital of Iowa Advisory Subcommittee 1997 PA/ARNP Credentials Committee (Chair) 1997 College of Medicine Research Advisory Committee State 1983-85 Texas Department of Health: Children's Speech, Hearing and Language Screening Advisory Committee 1984-89 Baylor College of Medicine Admissions Committee 1986-90 St. Luke's Episcopal Hospital: Medical Education, Publication and Research Committee 1986-90 Texas Children's Hospital Infections Committee 1987-90 Harris County Hospital District Accreditation Committee 1987-90 Harris County Hospital District Outpatient and Emergency Center Committee 1987-90 Harris County Hospital District Utilization Review Committee 1989-90 Baylor College of Medicine Curriculum Committee 1989-90 Baylor College of Medicine Student Financial Aid Committee 1989-90 Baylor College of Medicine Student Promotions and Academic Achievement Committee 1991 University of Iowa Medical Records Subcommittee 1992 Iowa Newborn Audiology Committee 1993 University of Iowa Ambulatory Surgery Committee 1994 University of Iowa Clinical Management Committee 1994 State of Iowa Committee on Deaf-Blind Screening
  • 37. Smith RJH 37 1994 State of Iowa Committee on Infant Hearing Screening National 1989-Audit Committee, American Society of Pediatric Otolaryngology 1990-National Advisory Committee for the Center for Hearing Loss in Children 1993-94 American Society of Pediatric Otolaryngology Program Chairman 1994-95 American Society of Pediatric Otolaryngology Program Chairman 1994-95 Home Study Course Chairman 1995-96 Organizing Committee, Association for Research in Otolaryngology 1995-98 Deafness Research Foundation Scientific Review Committee 1995-99 Long Range Planning Committee, Association for Research in Otolaryngology 1995-International Human Frontier Science Program, Review Committee 1995-Multi-Disciplinary Task Force on Recurrent Respiratory Papillomas 1995-NIDCD-Hearing Research Study Section (Ad hoc) 1996-Scope of Knowledge Task Force, American Board of Otolaryngology 1996-Pediatric Otolaryngology Committee - AAO-HNS, Inc. 1997-NIDCD R23 Special Emphasis Panel, Chair 1998-NIDCD PO1 Special Emphasis Panel, Chair 1997 NIDCD Working Group Co-Chair, Considerations for Developing and Implementing Genetic Diagnostic Tents for Hereditary Hearing Impairment and Other Communication Disorders 2001 1998 Board of Scientific Counselors of the National Institute on Deafness and Other Communication Disorders Hospital Affiliations 1983-90 Harris County Hospital District, Houston, Texas, Member, Active Staff, Otolaryngology Service 1983-90 St. Luke's Episcopal Hospital, Houston, Texas, Member, Active Staff, Otolaryngology Service 1983-90 The Methodist Hospital, Houston, Texas, Member, Courtesy Staff, Otolaryngology Service 1983-90 Texas Children's Hospital, Houston, Texas, Member, Active Staff, Otolaryngology Service 1983-90 Veteran's Administration Medical Center, Attending Physician, Otolaryngology Service, Texas, Crippled Children's Services Program 1983-90 Memorial Medical Center, Corpus Christi, Texas, Member, Academic Staff 1984-90 Texas Crippled Children's Services Program 1991- The University of Iowa Hospitals and Clinics, Professor, Department of Otolaryngology-Head and Neck Surgery Administrative Responsibilities 1987-90 Deputy Chief, Harris County Hospital District (Ben Taub General Hospital, Jefferson Davis Hospital) 1990- Director, Division of Pediatric Otolaryngology, University of Iowa 1990- Director, Molecular Otolaryngology Research Laboratories 1995- Vice Chairman, Department of Otolaryngology - Head and Neck Surgery, University of Iowa
  • 38. Smith RJH 38 2002- Surgical Director, Children’s Hospital of Iowa Professional Affiliations 1982 American Medical Association 1982-90 Texas Medical Association 1982-90 Harris County Medical Society 1982 American Academy of Otolaryngology - Head and Neck Surgery 1982 Deafness Research Foundation 1984 American Association for the History of Medicine 1984 International Society for the History of Otolaryngology 1984-90 Houston Pediatric Society 1985-90 Houston Otolaryngological Society 1985 American Society for Pediatric Otolaryngology (Charter Member) 1985 Society of University Otolaryngologists - Head and Neck Surgeons 1986 Texas Association of Otolaryngology - Head and Neck Surgery 1986 American Academy of Facial Plastic and Reconstructive Surgery 1987 Fellow, American College of Surgeons 1987 Fellow, American Academy of Pediatrics 1987 American Society of Human Genetics 1989 The American Laryngological, Rhinological and Otological Society (The Triological Society) 1991 Iowa Medical Society 1991 Johnson County Medical Society 1992 Research Association in Otolaryngology 1992 Brian F. McCabe Otorhinolaryngology Society 1992 Iowa Academy of Otolaryngology B. Clinical Activities Surgery Performed 1 day/week Clinic Patients seen 1½ days/week