PT - JOURNAL ARTICLE AU - Gülden Akdal AU - Koray Koçoğlu AU - Elçin Bora AU - Altuğ Koç AU - Ayfer Ülgenalp AU - Mithat Bedir AU - Rahmi Tümay Ala AU - Esra Battaloğlu AU - Günay Kırkım AU - İhsan Şükrü Şengün AU - Gábor Michael Halmágyi TI - Selective Bilateral Vestibular Neuropathy in a Turkish CMT1B Family With a Novel <em>MPZ</em> Mutation AID - 10.1212/CPJ.0000000000000930 DP - 2021 Apr 01 TA - Neurology: Clinical Practice PG - e129--e134 VI - 11 IP - 2 4099 - http://cp.neurology.org/content/11/2/e129.short 4100 - http://cp.neurology.org/content/11/2/e129.full AB - Purpose of Review To report the findings in 12 members over 3 generations of a family with dominantly inherited Charcot-Marie-Tooth disease (CMT1B) due to a novel MPZ mutation, who all had moderately severe selective impairment of vestibular function with normal hearing. Methods used were video head impulse testing of the function of all 6 semicircular canals, Romberg test on foam, nerve conduction studies, and whole exome and Sanger sequencing.Recent Findings All affected patients had a demyelinating neuropathy and a novel MPZ mutation: c.362A&gt;G (chr1: 161276584, p.D121G). All also had normal hearing for age but a moderately severe impairment of semicircular canal function and a positive Romberg test on foam.Summary Some CMT mutations can impair vestibular function, presumably because of a vestibular nerve involvement but spare hearing. In such patients, impairment of vestibular function and impairment of proprioception contribute to imbalance.