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Survey in to the Prevalence of Hearing Loss in Patients Diagnosed with Retinitis Pigmentosa

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Abstract

Sensorineural hearing loss is the most common disease associated with systemic retinitis pigmentosa (RP). We have conducted an epidemiological study to assess the correlation of age at onset of visual symptoms and hearing loss associated with RP. Epidemiological data was derived from a questionnaire-based study of patients who are registered members of the Japanese Retinitis Pigmentosa Society (n = 3200). The questionnaire was mailed to these patients in 2002, and information was requested regarding age at onset of visual disturbance, awareness of hearing loss and the presence of progressive hearing loss, age at onset of hearing loss, awareness of tinnitus, and history of audiometric examination and hearing aid usage. 26.1% of the questionnaires were returned, and data for 828 patients with RP diagnosed by an ophthalmologist were evaluated. Cochlear symptoms were reported by 356 patients (43.0% of the total population), with hearing loss in 29.5%, tinnitus in 31.5% and hearing loss and tinnitus in 39.3% of the 356 patients. Of these 356 patients, progressive hearing loss was reported by 44.9% and was independent of age at onset of cochlear symptoms. The mean age at onset of visual symptoms was higher for patients with progressive hearing loss, and a significant correlation was found between the age at onset of visual symptoms and hearing loss for patients who were older at onset of the symptoms (>30 years of age). Onset of hearing loss occurs later and hearing loss is also more progressive for patients with late onset of RP. This suggests that particular care regarding hearing loss is necessary for this patient population, and that cooperation between opthalmologists and otologists is required for diagnosis of RP-hearing impairment-associated syndromes in this group of patients.

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References

  1. A Graefe ParticleVon (1858) ArticleTitleVereinzelte Beobachtungen und Bemerkungen: Exceptionelles verrhalten des gesichtsfeldes bei pigmentenartung der netzhaut Von Graefe’s Arch Ophthalmol 4 250–253

    Google Scholar 

  2. CH Usher (1914) ArticleTitleOn the inheritance of retinitis pigmentosa, with␣notes of cases R Lond Ophthal Hosp Rep 19 130–256

    Google Scholar 

  3. M Haim (1992) ArticleTitlePrevalence of retinitis pigmentosa and allied disorders in Denmark. II: Systemic involvement and age at onset Acta Ophthalmologica 70 417–426 Occurrence Handle1:STN:280:ByyD3svhvFA%3D Occurrence Handle1414285

    CAS  PubMed  Google Scholar 

  4. M Vernon (1969) ArticleTitleUsher syndrome deafness and progressive blindness: Clinical cases, prevention, theory and literature survey J Chronic Dis 22 133–151 Occurrence Handle10.1016/0021-9681(69)90055-1 Occurrence Handle1:STN:280:CCaB1MvmtVQ%3D Occurrence Handle4897966

    Article  CAS  PubMed  Google Scholar 

  5. RJH Smith CI Berlin JF Hejtmancik et al. (1994) ArticleTitleClinical diagnosis of the usher syndromes Am J Med Genet 50 32–38 Occurrence Handle10.1002/ajmg.1320500107 Occurrence Handle1:STN:280:ByuB3M3pvVI%3D Occurrence Handle8160750

    Article  CAS  PubMed  Google Scholar 

  6. ET Tsilou BI Rubin RC Caruso et al. (2002) ArticleTitleUsher syndrome clinical types I and II: Could ocular symptoms and signs differentiate between the two types? Acta Ophthalmol Scand 80 196–201 Occurrence Handle10.1034/j.1600-0420.2002.800215.x Occurrence Handle11952489

    Article  PubMed  Google Scholar 

  7. A Nuutila (1970) ArticleTitleDystrophia retinae pigmentosa-dysacusis syndrome (DRD): A study of the Usher or Hallgren syndrome J Genet Hum 18 57–58 Occurrence Handle1:STN:280:CS6C1cfoslw%3D Occurrence Handle5516287

    CAS  PubMed  Google Scholar 

  8. M Haim (2002) ArticleTitleThe epidemiology of retinitis pigmentosa in Denmark Acta Ophthalmol Scand Suppl 233 1–34 Occurrence Handle11921605

    PubMed  Google Scholar 

  9. M Hayakawa K Fujiki A Kanai et al. (1997) ArticleTitleMulticenter genetic study of retinitis pigmentosa in Japan II: Prevalence of autosomal recessive retinitis pigmentosa Jpn J Ophthalmol 41 7–11 Occurrence Handle1:STN:280:ByiB1M3mslQ%3D Occurrence Handle9147181

    CAS  PubMed  Google Scholar 

  10. L Pakarinen K Tuppurainen P Laippala M Mantyjarvi H Puhakka (1996) ArticleTitleThe ophthalmological course of Usher syndrome type III Int Ophthalmol 19 307–311 Occurrence Handle1:STN:280:ByiD3MfitVQ%3D

    CAS  Google Scholar 

  11. RJE Pennings PLM Huygen MD Weston et al. (2003) ArticleTitlePure tone hearing thresholds and speech recognition scores in Dutch patients carrying mutations in the USH2A gene Otology & Neurotology 24 58–63

    Google Scholar 

  12. Davenport SLH, Omen GS. The heterogeneity of Usher syndrome, publication 426. Amsterdam, Excerpta Medica Foundation, International Congress Series, abstract 215, 1977: 87–88.

  13. T Rosenberg M Haim AM Hauch et al. (1997) ArticleTitleThe prevalence of Usher syndrome and other retinal dystrophy-hearing impairment associations Clin Genet 51 314–321 Occurrence Handle1:STN:280:ByiA2MrmvFw%3D Occurrence Handle9212179

    CAS  PubMed  Google Scholar 

  14. CI Hope S Bundey D Proops et al. (1997) ArticleTitleUsher syndrome in the city of Birmingham prevalence and clinical classification. Br J Ophthalmol 81 46–53 Occurrence Handle1:STN:280:ByiB1c7htVQ%3D Occurrence Handle9135408

    CAS  PubMed  Google Scholar 

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Correspondence to Satoshi Iwasaki.

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Iwasaki, S., Maruyama, Y., Hotta, Y. et al. Survey in to the Prevalence of Hearing Loss in Patients Diagnosed with Retinitis Pigmentosa. Int Ophthalmol 25, 277–282 (2004). https://doi.org/10.1007/s10792-005-2035-y

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  • DOI: https://doi.org/10.1007/s10792-005-2035-y

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