A dog was evaluated for rapidly progressive mentation change, ataxia, and tetraparesis. The dog's neurological status deteriorated drastically. It became comatose with bilateral mydriasis, and the pupillary light reflex was absent. An anti-inflammatory dose of methylprednisolone was administered, and temporary stabilization of neurological status was achieved. MRI findings were suggestive of ventriculitis and meningoencephalitis originating from the left tympanic cavity. A gadolinium leakage phenomenon was noted, likely resulting from severe damage to the blood-cerebrospinal fluid barrier during the inflammatory process. Analysis of the cerebrospinal fluid and materials in the left tympanic cavity further confirmed the diagnosis. Following surgical and antibiotic treatment, the dog recovered well with only a mild residual head tilt. Seven months after surgery, the dog had a recurrent infection of the left tympanic cavity without intracranial involvement. A second surgery led to an uneventful recovery, and the dog was clinically normal except for a mild head tilt 3 yr after the initial presentation. This is the first report describing ventriculitis associated with otogenic meningoencephalitis in dogs and a gadolinium leakage phenomenon displayed on MRI. The long-term outcome of ventriculitis-complicated otogenic meningoencephalitis in dogs could be satisfied with prompt diagnosis and treatment.