Hearing outcomes of cochlear implantation in IP-II (Mondini Dysplasia) – A systematic review.
Hearing outcomes of cochlear implantation in IP-II (Mondini Dysplasia) – A systematic review.
Objectives: Incomplete Partition type II (IP-II), or Mondini dysplasia, accounts for half of congenital cochlear malformations and results in profound sensorineural hearing loss. Cochlear implantation restores hearing in patients with IP-II however low numbers mean reporting of auditory performance outcomes is limited. Here we review English language articles between 2010 and 2025 reporting hearing outcomes following cochlear implantation in individuals IP-II. Results: Categories of Auditory Performance (CAP), Speech Intelligibility Rating (SIR) and parental reports of cochlear implantation in 731 individuals, across 20 studies, with a follow-up of 1–3 years, met the inclusion criteria. Findings suggest that auditory perception and speech develop more slowly in people with IP-II compared to controls but there was no difference between groups by 1–2 years post-implantation. Discussion: Most patients with IP-II have better hearing with a cochlear implant than before. However, there is limited evidence that this is comparable to those with normal middle ear anatomy. Findings were typically derived from questionnaires with small cohorts and short follow-up. Conclusion: Patient counselling for people IP-II should identify that prediction of hearing outcomes is uncertain. Informed prognostication of timing of surgery and hearing outcomes after cochlear implantation in IP-II needs further study with long-term follow-up including surgical and hearing outcome measures.
Hearing loss, IP-2, Mondini dysplasia, clinical outcome, cochlear implant, hearing performance, inner ear malformation, systematic review
149-156
Findlay, Callum Andrew
a8f4f69f-e3bc-4a5f-b1d8-c5e38ba0d00e
Arthur, Emelyne
019128c3-001a-43fa-bf8c-b43590315efd
Hough, Kate
81d8630c-6e02-4bea-858a-377717476f6e
Grasmeder, Mary
206e6b44-d1cd-43f5-99ac-588ab02d44ef
Newman, Tracey
322290cb-2e9c-445d-a047-00b1bea39a25
Findlay, Callum Andrew
a8f4f69f-e3bc-4a5f-b1d8-c5e38ba0d00e
Arthur, Emelyne
019128c3-001a-43fa-bf8c-b43590315efd
Hough, Kate
81d8630c-6e02-4bea-858a-377717476f6e
Grasmeder, Mary
206e6b44-d1cd-43f5-99ac-588ab02d44ef
Newman, Tracey
322290cb-2e9c-445d-a047-00b1bea39a25
Findlay, Callum Andrew, Arthur, Emelyne, Hough, Kate, Grasmeder, Mary and Newman, Tracey
(2026)
Hearing outcomes of cochlear implantation in IP-II (Mondini Dysplasia) – A systematic review.
Cochlear Implants International, .
(doi:10.1080/14670100.2026.2635187).
Abstract
Objectives: Incomplete Partition type II (IP-II), or Mondini dysplasia, accounts for half of congenital cochlear malformations and results in profound sensorineural hearing loss. Cochlear implantation restores hearing in patients with IP-II however low numbers mean reporting of auditory performance outcomes is limited. Here we review English language articles between 2010 and 2025 reporting hearing outcomes following cochlear implantation in individuals IP-II. Results: Categories of Auditory Performance (CAP), Speech Intelligibility Rating (SIR) and parental reports of cochlear implantation in 731 individuals, across 20 studies, with a follow-up of 1–3 years, met the inclusion criteria. Findings suggest that auditory perception and speech develop more slowly in people with IP-II compared to controls but there was no difference between groups by 1–2 years post-implantation. Discussion: Most patients with IP-II have better hearing with a cochlear implant than before. However, there is limited evidence that this is comparable to those with normal middle ear anatomy. Findings were typically derived from questionnaires with small cohorts and short follow-up. Conclusion: Patient counselling for people IP-II should identify that prediction of hearing outcomes is uncertain. Informed prognostication of timing of surgery and hearing outcomes after cochlear implantation in IP-II needs further study with long-term follow-up including surgical and hearing outcome measures.
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Accepted/In Press date: 17 February 2026
e-pub ahead of print date: 3 March 2026
Additional Information:
Publisher Copyright:
© 2026 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group.
Keywords:
Hearing loss, IP-2, Mondini dysplasia, clinical outcome, cochlear implant, hearing performance, inner ear malformation, systematic review
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Local EPrints ID: 510345
URI: http://eprints.soton.ac.uk/id/eprint/510345
ISSN: 1467-0100
PURE UUID: c63f489c-cc76-4560-b1f9-c447a9484b2d
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Date deposited: 26 Mar 2026 18:01
Last modified: 27 Mar 2026 03:09
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Author:
Callum Andrew Findlay
Author:
Emelyne Arthur
Author:
Kate Hough
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