Hearing loss, tinnitus, and vertigo are very common complaints in otolaryngology practice. Here, we describe spontaneous intracranial hypotension (SIH) as a curable reason of hearing loss, tinnitus, and vertigo. A 29-year-old woman presented to the emergency room with nausea, dizziness, vertigo, instability, hearing loss, tinnitus, and neck and back pain. Cranial computed tomography, magnetic resonance imaging (MRI), and lumbar puncture were performed. The patient stated that the hearing loss and tinnitus became worse after effort or standing for prolonged times. Therefore, we performed audiogram in sitting and standing positions. The tinnitus severity index was used to evaluate tinnitus. Lumbar puncture revealed no cerebrospinal fluid, and cerebrospinal fluid could be obtained by aspiration. Cranial MRI showed dural thickness and venous engorgement in the internal acoustic canals bilaterally. Audiography showed worse hearing capacity in standing position than in sitting position and revealed especially low-frequency hearing loss bilaterally. The patient's tinnitus severity index was 48 of 60. The patient was diagnosed as having SIH and treated with autologous blood punch. Cranial MRI and audiogram were normal after the treatment. The patient had no tinnitus after the treatment. Spontaneous intracranial hypotension, which may cause Meniere syndrome-like symptoms, is a curable reason of hearing loss, tinnitus, and vertigo. In addition, the fluctuation of the hearing loss with positional changes supports the use of positional audiometry when evaluating hearing loss-related SIH. Venous engorgement in the internal acoustic canal may be related to the symptoms.