Infantswithcongenitaldeafnesscausedbyseverebilateralinnerearmalformationsfrequentlysufferfromseverehearinglossandpoorbalance.Unfortunately,theuseofhearingaidsisusuallyineffectiveinrecoveringhearing,necessitatingcochlearimplants.Wereportacaseofa6-year-oldboywithcongenitaldeafnessandbilateralinnerearmalformations(rightside,incompletepartitiontypeI[IP-I];leftside,commoncavitydeformity).Hearingaidshadaremarkableeffectinthispatient,enablingsufficientandfavorablehearingrecoverysuchastoallowthepatienttoengageindailyconversations.Per-rotatorynystagmuswasrecordedonanelectronystagmogramforbothrightandleftrotationsinadampedrotationalchairtest.Itisrarefordeafchildrenwithseverebilateralinnerearmalformationtodemonstratefavorabledevelopmentinhearingandgoodequilibriumfunction.OurfindingssuggestthatauditoryevestibularhaircellsinthispatientmayhavebeenpartiallypreserveddespiteIP-Iintherightearandcommoncavitydeformityoftheleftear.
