Cochlear implantation and Cogan syndrome.
OBJECTIVE
To evaluate outcomes and issues pertaining to cochlear implantation in a group of subjects affected by Cogan syndrome.


METHODS
Prospective cohort.


METHODS
Department of Ophthalmology and Otorhinolaryngology, University of Parma.


METHODS
Five postlingually deafened adults suffering from a typical form of Cogan syndrome who underwent cochlear implantation.


METHODS
Benefit from cochlear implantation as measured by word and everyday sentence recognition tests. Surgical issues and postoperative complications were also evaluated.


RESULTS
In two cases, intracochlear electrodes were inserted into the scala vestibuli because of the ossification of the scala tympani. Two patients experienced a recurrence of keratitis the day after surgery. To date, with a follow-up of 1 to 4 years, no patient has experienced flap complications or other local or systemic complications. At the 12-month postoperative evaluation, all patients had gained useful open-set speech perception, achieving a mean score of 91% and 95% on word and everyday sentence recognition tests, respectively.


CONCLUSIONS
Patients deafened by Cogan syndrome demonstrated high levels of speech understanding after undergoing cochlear implantation. Obliteration of the cochlea may complicate electrode implantation, requiring modifications of the surgical technique. Stress consequent to the surgical procedure may instigate an acute phase of the basic illness.
Pasanisi
Enrico
E
Department of Ophthalmology and Otorhinolaryngology, University of Parma, Parma, Italy. enrico.pasanisi@unipr.it
Vincenti
Vincenzo
V
Bacciu
Andrea
A
Guida
Maurizio
M
Berghenti
Teresa
T
Barbot
Anna
A
Orsoni
Jelka G
JG
Bacciu
Salvatore
S
D000328:Adult; D015834:Cochlear Diseases / Q000150:complications; D019929:Cochlear Implantation / Q000379:methods; D015331:Cohort Studies; D003142:Communication; D003638:Deafness / Q000150:complications* / Q000503:physiopathology / Q000523:psychology / Q000601:surgery*; D003858:Dependency, Psychological; D004493:Education; D005128:Eye Diseases / Q000150:complications*; D005260:Female; D005639:Frustration; D006801:Humans; D007398:Interpersonal Relations; D008297:Male; D009999:Ossification, Heterotopic / Q000150:complications; D011446:Prospective Studies; D035583:Rare Diseases; D013067:Speech Perception; D013577:Syndrome; D016896:Treatment Outcome