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CEACAM16 is a non-collagenous protein of the tectorial membrane, an extracellular structure of the cochlea essential for normal hearing. Dominant and recessive mutations in  CEACAM16  have been reported to cause postlingual and progressive forms of deafness in humans. In a previous study of young  Ceacam16 βgal/βgal   null mutant mice on a C57Bl/6J background, the incidence of spontaneous otoacoustic emissions (SOAEs) was greatly increased relative to  Ceacam16 +/+   and  Ceacam16 +/βgal   mice, but auditory brain-stem responses (ABRs) and distortion product otoacoustic emissions (DPOAEs) were near normal, indicating auditory thresholds were not significantly affected. To determine if the loss of CEACAM16 leads to hearing loss at later ages in this mouse line, cochlear structure and auditory function were examined in  Ceacam16 +/+ , Ceacam16 +/βgal   and  Ceacam16 βgal/βgal   mice at 6 and 12 months of age and compared to that previously described at 1 month. Analysis of older  Ceacam16 βgal/βgal   mice reveals a progressive loss of matrix from the core of the tectorial membrane that is more extensive in the apical, low-frequency regions of the cochlea. In  Ceacam16 βgal/βgal   mice at 6–7 months, the DPOAE magnitude at 2f1-f2 and the incidence of SOAEs both decrease relative to young animals. By ∼12 months, SOAEs and DPOAEs are not detected in  Ceacam16 βgal/βgal   mice and ABR thresholds are increased by up to ∼40 dB across frequency, despite a complement of hair cells similar to that present in  Ceacam16 +/+   mice. Although SOAE incidence decreases with age in  Ceacam16 βgal/βgal   mice, it increases in aging heterozygous  Ceacam16 +/βgal   mice and is accompanied by a reduction in the accumulation of CEACAM16 in the tectorial membrane relative to controls. An apically-biased loss of matrix from the core of the tectorial membrane, similar to that observed in young  Ceacam16 βgal/βgal   mice, is also seen in  Ceacam16 +/+   and  Ceacam16 +/βgal   mice, and other strains of wild-type mice, but at much later ages. The loss of  Ceacam16  therefore accelerates age-related degeneration of the tectorial membrane leading, as in humans with mutations in  CEACAM16 , to a late-onset progressive form of hearing loss.

frontiers in molecular neuroscience

Accelerated Age-Related Degradation of the Tectorial Membrane in the Ceacam16βgal/βgal Null Mutant Mouse, a Model for Late-Onset Human Hereditary Deafness DFNB113