Results from Four Cochlear Implant Patients with Usher's Syndrome

Abstract

Individual results are presented of 4 patients with Usher's syndrome type 1 who received a cochlear implant. Both single-channel and multichannel implants were used. Because of implant failure, one of the single-channel systems was replaced by a Nucleus multichannel system. Results are compared to the results of 5 other prelingually deaf cochlear implant users. The performance of the patients with Usher's syndrome on suprasegmental and segmental speech perception tests and on a connected discourse tracking task did not differ significantly from the performance of the other prelingually deaf patients. A significant improvement over time was found at the suprasegmental level for the combined group of Usher's and other patients. No obvious differences were found between the scores from the patients with a single-channel and the patients with a multichannel device. The rehabilitation of the Usher's patients required very little extra effort in comparison with that of the other prelingually deaf patients; all patients reported considerable advantages in hearing abilities and social life.

Keywords

cochlear implants speech perception Usher's syndrome

Get full access to this article

View all access options for this article.

References

Gantz

Murray

Tye N

Tyler

. Word recognition performance with single-channel and multichannel cochlear implants. Am J Otol 1989; 10: 91–4.

Dowell

Mecklenburg

Clark

. Speech recognition for 40 patients receiving multichannel cochlear implants. Arch Otolaryngol Head Neck Surg 1986; 112: 1054–9.

Berliner

Luxford

House

. Cochlear implants: 1981–1985. Am J Otol 1985; 2: 173–86.

Clark

Busby

Roberts

. Preliminary results for the Cochlear Corporation multielectrode intracochlear implant in six prelingually deaf patients. Am J Otol 1987; 8: 234–9.

Tong

Busby

Clark

. Perceptual studies on cochlear implant patients with early onset of profound hearing impairment prior to normal development of auditory, speech and language skills. J Acoust Soc Am 1988; 84: 951–62.

Dawson

Blamey

Rowland

. Cochlear implants in children, adolescents, and prelinguistically deafened adults: Speech perception. J Speech Hear Res 1992; 35: 401–17.

Waltzman

Cohen

Shapiro

. Use of a multichannel cochlear implant in the congenitally and prelingually deaf population. Laryngoscope 1992; 102: 395–9.

Vernon

. Usher's syndrome: Deafness and progressive blindness. Clinical cases, prevention, theory and literature survey. J Chronic Dis 1969; 22: 133–51.

Eisenberg

. Use of the cochlear implant by the prelingually deaf. Ann Otol Rhinol Laryngol 1982; 91(suppl 91): 62–6.

10.

Martin

. Speech recognition by a deaf-blind multichannel cochlear implant patient. Ear Hear 1988; 9: 70–4.

11.

Van den Broek

Admiraal

. Selection of patients for a cochlear implant. Acta Otorhinolaryngol Belg 1991; 45: 243–6.

12.

Van den Broek

Coninx

Mens

Admiraal

Brokx

. Temporary placement of electrodes as part of a selection procedure for cochlear implants. In: Charachon

Ibañez

Garcia E

, eds. Long term results and indications in otology and otoneurosurgery. POL 051–3. Proceedings of the Politzer Society Conference in Ibiza, 1989 and Courchevel, 1990. Amsterdam, the Netherlands: Kugler Publications, 1990: 1–3.

13.

Erber

Alencewicz

. Audiologic evaluation of deaf children. J Speech Hear Disord 1976; 41: 256–67.

14.

Brokx

JPL

Beynon

van Durme

Timmermans

. Ontwikkeling van Nederlandstalige auditieve tests. Logopedie Foniatrie 1987; 59: 186–90.

15.

Beynon

Brokx

JPL

Mens

LHM

van den Broek

. De eerste resultaten met een elektrische binnenoorprothese. Logopedie Foniatrie 1990; 62: 113–6.

16.

Owens

Kessler

Raggio

. Analysis and revision of the Minimal Auditory Capabilities (MAC) battery. Ear Hear 1985; 6: 280–90.

17.

Tyler

Preece

Lowder

. The Iowa cochlear-implant test battery. Iowa City, Iowa: Department of Otolaryngology—Head and Neck Surgery, University of Iowa, 1983.

18.

De Filippo

Scott

. A method for training and evaluating the reception of ongoing speech. J Acoust Soc Am 1987; 63: 1186–92.

19.

Morgan

Dirks

Bower

. Suggested threshold sound pressure levels for frequency modulated (warble) tones in the sound field. J Speech Hear Disord 1979; 44: 37–54.

20.

Tye-Murray

Tyler

. A critique of continuous discourse tracking as a test procedure. J Speech Hear Disord 1988; 53: 226–31.

21.

Miyamoto

Osberger

Robbins

Myres

Kessler

Pope

. Longitudinal evaluation of communication skills of children with single- or multichannel cochlear implants. Am J Otol 1992; 13: 215–22.

22.

Doyle

Pijl

Noel

. The cochlear implant: A comparison of single-channel and multichannel results. J Otolaryngol 1991; 20: 204–8.

23.

Tyler

Moore

Kuk

. Performance of some of the better cochlear implant patients. J Speech Hear Res 1989; 32: 887–911.