Outcomes of cochlear implantation in Usher syndrome: a systematic review

Purpose This study is a systematic review of the literature which seeks to evaluate auditory and quality of life (QOL) outcomes of cochlear implantation in patients with Usher syndrome. Methods Systematic review of studies indexed in Medline via PubMed, Ovid EMBASE, Web of Science, CENTRAL and clinicaltrials.gov was performed up to March 9th 2022, conducted in accordance with the PRISMA statement. Patient demographics, comorbidity, details of cochlear implantation, auditory, and QOL outcomes were extracted and summarized. Results 33 studies reported over 217 cochlear implants in 187 patients with Usher syndrome, comprising subtypes 1 (56 patients), 2 (9 patients), 3 (23 patients), and not specified (99 patients). Auditory outcomes included improved sound detection, speech perception, and speech intelligibility. QOL outcomes were reported for 75 patients, with benefit reported in the majority. Conclusions Many patients with Usher syndrome develop improved auditory outcomes after cochlear implantation with early implantation being an important factor. Supplementary Information The online version contains supplementary material available at 10.1007/s00405-023-08304-2.


Speech perception
With hearing aids.Scores presented for patients 6, 7, 10, in order.N.T. = not tested.
Case 1 can identify words based on stress pattern but has not yet reached a passing score on spectral tasks.
Case 2 can identify words based on stress pattern when the set size is controlled but has no consistent word identification based on spectral content.

Other
Both attended mainstream school with an interpreter.
Both are full-time CI users.

Speech perception
Can identify LING sounds across the speech frequency range (0.3-4 kHz).

Other
Can follow two-or three-item instructions.
Improvement in general self-confidence, perception of environmental sounds and lip-reading skill.

Speech perception
Improved pattern perception, beginning to develop closed-set skills for familiar names.

Other
Greater awareness of environmental sounds.Increased anxiety when the implant was not turned on.

Speech perception
No measurable word recognition threshold.

Other
Patient reported feeling less dependent on relatives, more confident, social and happy.

24)
Speech perception CAP score: 0 for all patients.
CAP score (mean): 3. General working memory (WM) similar to HA and NH, better than CI.Visual WM scores over a wide range of performance.
Phonological WM generally worse than HA and NH, similar to or better than CI. 2 of 7 scored similar to or better than NH and HA.
Performance on phonological and lexical skill tasks tended to be in line with expected performance for NH and HA groups for children implanted before age 2 years, but closer to that of nonsyndromic CI users when implanted at later ages.123  congenital in 8 cases and progressive in one case.
One patient had mild cognitive delay.

Speech perception
Geers

Speech intelligibility
Parents report 10 functional words and a wide variety of sounds.