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J R Soc Med. 1987 January; 80(1): 17–20.
PMCID: PMC1290625

Retinitis pigmentosa and deafness.


Seventeen patients with retinitis pigmentosa (RP) have been investigated audiologically. Of 9 found to have a significant hearing loss, 6 were examples of Usher's syndrome; these patients had a cochlear pattern of hearing loss. The other 3 were examples of Senior's syndrome, Kearne-Sayre syndrome and Lawrence-Moon-Biedle syndrome respectively. Two of these patients had absent stapedius reflexes. It is suggested that patients with different RP-deafness syndromes may have lesions in different parts of the auditory system.

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Selected References

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  • Beatty CW, McDonald TJ, Colvard DM. Usher's syndrome with unusual otologic manifestations. Mayo Clin Proc. 1979 Aug;54(8):543–546. [PubMed]
  • Gorlin RJ, Tilsner TJ, Feinstein S, Duvall AJ., 3rd Usher's syndrome type III. Arch Otolaryngol. 1979 Jun;105(6):353–354. [PubMed]
  • Hurley RM, Dery P, Norady MB, Drummond KN. The renal lesion of the Laurence-Moon-Biedl syndrome. J Pediatr. 1975 Aug;87(2):206–209. [PubMed]
  • ALSTROM CH, HALLGREN B, NILSSON LB, ASANDER H. Retinal degeneration combined with obesity, diabetes mellitus and neurogenous deafness: a specific syndrome (not hitherto described) distinct from the Laurence-Moon-Bardet-Biedl syndrome: a clinical, endocrinological and genetic examination based on a large pedigree. Acta Psychiatr Neurol Scand Suppl. 1959;129:1–35. [PubMed]
  • Goldstein JL, Fialkow PJ. The Alström syndrome. Report of three cases with further delineation of the clinical, pathophysiological, and genetic aspects of the disorder. Medicine (Baltimore) 1973 Jan;52(1):53–71. [PubMed]
  • KEARNS TP, SAYRE GP. Retinitis pigmentosa, external ophthalmophegia, and complete heart block: unusual syndrome with histologic study in one of two cases. AMA Arch Ophthalmol. 1958 Aug;60(2):280–289. [PubMed]
  • Bateman JB, Riedner ED, Levin LS, Maumenee IH. Heterogeneity of retinal degeneration and hearing impairment syndromes. Am J Ophthalmol. 1980 Dec;90(6):755–767. [PubMed]
  • Olson W, Engel WK, Walsh GO, Einaugler R. Oculocraniosomatic neuromuscular disease with "ragged-red" fibers. Arch Neurol. 1972 Mar;26(3):193–211. [PubMed]
  • Egger J, Lake BD, Wilson J. Mitochondrial cytopathy. A multisystem disorder with ragged red fibres on muscle biopsy. Arch Dis Child. 1981 Oct;56(10):741–752. [PMC free article] [PubMed]
  • Goode RL, Rafaty FM, Simmons FB. Hearing loss in retinitis pigmentosa. Pediatrics. 1967 Nov;40(5):875–880. [PubMed]
  • Davenport SL, O'Nuallain S, Omenn GS, Wilkus RJ. Usher syndrome in four hard-of-hearing siblings. Pediatrics. 1978 Oct;62(4):578–583. [PubMed]
  • Belal A., Jr Usher's syndrome. (Retinitis pigmentosa and deafness). A temporal bone report. J Laryngol Otol. 1975 Feb;89(2):175–181. [PubMed]
  • Kumar A, Fishman G, Torok N. Vestibular and auditory function in Usher's syndrome. Ann Otol Rhinol Laryngol. 1984 Nov-Dec;93(6 Pt 1):600–608. [PubMed]
  • DE ROBERTIS E. Morphogenesis of the retinal rods; an electron microscope study. J Biophys Biochem Cytol. 1956 Jul 25;2(4 Suppl):209–218. [PMC free article] [PubMed]
  • Arden GB, Fox B. Increased incidence of abnormal nasal cilia in patients with retinitis pigmentosa. Nature. 1979 Jun 7;279(5713):534–536. [PubMed]
  • SENIOR B, FRIEDMANN AI, BRAUDO JL. Juvenile familial nephropathy with tapetoretinal degeneration. A new oculorenal dystrophy. Am J Ophthalmol. 1961 Nov;52:625–633. [PubMed]
  • LOKEN AC, HANSSEN O, HALVORSEN S, JOLSTER NJ. Hereditary renal dysplasia and blindness. Acta Paediatr. 1961 Mar;50:177–184. [PubMed]
  • Shemen LJ, Mitchell DP, Farkashidy J. Cockayne syndrome--an audiologic and temporal bone analysis. Am J Otol. 1984 Apr;5(4):300–307. [PubMed]

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