Mixed conductive and sensorineural hearing loss in LP/J mice

• Published in: Hearing research Vol. 28; no. 2; pp. 227 - 236
• Main Authors: Steel, Karen P., Moorjani, Padma, Bock, Gregory R.
• Format: Journal Article
• Language: English
• Published: Amsterdam Elsevier B.V 1987; Elsevier
• Subjects: Action Potentials; Air-bone gap; Animals; Biological and medical sciences; Bone and Bones - pathology; Cochlea - physiopathology; Conductive hearing loss; Ear, auditive nerve, cochleovestibular tract, facial nerve: diseases, semeiology; Hearing Loss - physiopathology; Hearing Loss, Conductive - pathology; Hearing Loss, Conductive - physiopathology; Hearing Loss, Sensorineural - pathology; Hearing Loss, Sensorineural - physiopathology; LP/J mouse; Medical sciences; Mice; Mice, Inbred CBA; Mice, Inbred Strains; Middle-ear anomaly; Organ of Corti - pathology; Ossicular lesion; Otorhinolaryngology. Stomatology; Otosclerosis; Sensory Thresholds; Middle-ear anomaly; Air-bone gap; Conductive hearing loss; Ossicular lesion; LP/J mouse; Otosclerosis; Animal model; Vertebrata; Experimental disease; Mixed hearing loss; Mammalia; Mouse; Ear disease; Rodentia; ENT disease; Hereditary
• ISSN: 0378-5955; 1878-5891
• DOI: 10.1016/0378-5955(87)90051-7

Air and bone conduction thresholds for the detection of a compound action potential response were measured in mice of the LP/J inbred strain, which has been proposed as a possible model for human otosclerosis. Thresholds were compared with control data from CBA/Ca mice. Evidence of a mixed sensorineural and conductive hearing loss was obtained in LP/J mice. Few signs of hair cell degeneration or middle-ear bony lesions were found in 20-day old mice, although they generally showed raised thresholds to both air and bone conducted stimuli. The reason for the observed sensorineural component of the hearing loss at this age is not clear, since endocochlear potentials were normal in mice of this strain. By 225 days of age, hair cell loss was extensive and there was clearly excess bone growth in all middle-ear specimens studied, particularly on the incus and the cochlear wall facing the middle ear. Microbiological analysis of LP/J specimens revealed no evidence of a middle-ear infection specific to these mice which might explain the pathology.