X-linked Malformation and Cochlear Implantation.

Abstract

OBJECTIVE:

To evaluate if cochlear implantation is safe and constitutes an option for hearing rehabilitation of children with x-linked inner ear malformation.

STUDY DESIGN:

Retrospective patient review in combination with a multidisciplinary follow-up.

SETTING:

Tertiary referral hospital and cochlear implant program.

PATIENTS:

Ten children with severe-profound mixed hearing loss and radiological findings consistent with Incomplete Partition type 3 cochlear malformation received cochlear implants during the years 2007 to 2015. Nine of the children had a mutation affecting the gene POU3F4 on Xq21.

INTERVENTION:

Cochlear implantation.

MAIN OUTCOME MEASURES:

Surgical events, intraoperative measures and electrical stimulation levels, hearing and spoken language abilities.

RESULTS:

In all, 15 cochlear implantations were performed. In three cases the electrode was found to be in the internal auditory canal on intraoperative x-ray and repositioned successfully. One child had a postoperative rhinorrhea confirmed to be cerebrospinal fluid but this resolved on conservative treatment. No severe complications occurred. Postoperative electrical stimulation levels were higher in 9 of 10 children, as compared with typically reported average levels in patients with a normal cochlea. Eight patients developed spoken language to various degrees while two were still at precommunication level. However, speech recognition scores were lower than average pediatric cases.

CONCLUSION:

Cochlear implantation is a safe procedure for children with severe-profound mixed hearing loss related to POU3F4 mutation inner ear malformation. The children develop hearing and spoken language but outcome is below average for pediatric CI recipients.

• Authors: Henrik Smeds, Jeremy Wales, Filip Asp, Ulrika Löfkvist, Babak Falahat, Britt-Marie Anderlid, Lena Anmyr, Eva Karltorp
• Journal: Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology (Otol Neurotol) Vol. 38 Issue 1 Pg. 38-46 (01 2017) ISSN: 1537-4505 [Electronic] United States
• PMID: 27779564 (Publication Type: Journal Article)

Chemical References

• POU Domain Factors
• POU3F4 protein, human

Topics

• Child
• Child, Preschool
• Chromosomes, Human, X
• Cochlea (surgery)
• Cochlear Implantation (adverse effects, methods)
• Cochlear Implants
• Ear, Inner (abnormalities, surgery)
• Female
• Genetic Diseases, X-Linked
• Hearing Loss, Sensorineural (congenital, genetics, surgery)
• Hearing Tests
• Humans
• Male
• Mutation
• POU Domain Factors (genetics)
• Postoperative Complications (epidemiology)
• Retrospective Studies
• Treatment Outcome