Outcomes and special considerations of cochlear implantation in waardenburg syndrome.

Abstract

OBJECTIVES:

The objective of this study was a state-of-the-art analysis of cochlear implantation in patients with Waardenburg syndrome (WS).

PATIENTS:

Twenty-five patients with WS treated with cochlear implants in our department from 1990 to 2010.

INTERVENTIONS:

The 25 patients with WS underwent 35 cochlear implantations.

MAIN OUTCOME MEASURES:

Hearing outcome was evaluated using HSM sentence test in 65 dB in quiet, Freiburg Monosyllabic Test, and categories of auditory performance for children and compared with that of a control group. Anatomic abnormalities of the inner ear were examined using magnetic resonance imaging and computed tomography of the temporal bones.

RESULTS:

The mean follow-up time was 8.3 years (range, 0.3-18.3 yr). The majority achieved favorable postimplantation performance with mean HSM scores of 75.3% (range, 22.6%-99%) and Freiburg Monosyllabic Test scores of 67.8% (range, 14%-95%). However, in 4 cases, the results were less satisfactory. The comparison with the control group did not reveal any statistical significance (p = 0.56). In 6 patients (24%), behavioral disorders caused temporary difficulties during the rehabilitation procedure. Except of isolated large vestibule in 1 patient, the radiological assessment of the 50 temporal bones did not reveal any temporal bone abnormalities.

CONCLUSION:

Most patients with WS performed well with cochlear implants. However, WS is related to behavioral disorders that may cause temporary rehabilitation difficulties. Finally, temporal bone malformations that could affect cochlear implantation are notcharacteristic of WS.

• Authors: Georgios Kontorinis, Thomas Lenarz, Alexandros Giourgas, Martin Durisin, Anke Lesinski-Schiedat
• Journal: Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology (Otol Neurotol) Vol. 32 Issue 6 Pg. 951-5 (Aug 2011) ISSN: 1537-4505 [Electronic] United States
• PMID: 21512421 (Publication Type: Journal Article)

Topics

• Adolescent
• Child
• Child, Preschool
• Cochlear Implantation
• Cochlear Implants
• Female
• Follow-Up Studies
• Hearing Loss, Sensorineural (surgery)
• Humans
• Infant
• Male
• Patient Satisfaction
• Treatment Outcome
• Waardenburg Syndrome (surgery)