For some, permanent damage occurs, and they may be left with long-term hearing loss, hearing distortion, tinnitus, and/or a feeling of fullness in the affected ear(s).A study looking at spiral ganglion cell counts compared to hair cell counts in the inner ear of patients who had Menieres disease found that they maintained more hair cells than spinal ganglion cells. Thus, it could be possible that hydrops affects auditory nerves more than hair cells. In contrast, a 2021 article by Richard Gacek posits that the hearing loss is actually caused by toxic nucleic acids that are released to the outer hair cells: "Since the outer hair cells (OHC) are freely surrounded by perilymph, their walls and nerve terminals are also bathed in this fluid. The few type-II spiral ganglion cells in contact with the OHC are unlikely to play a significant role in hearing loss because of their low numbers and the lack of a known connection to the central auditory pathway." Symptoms
Cochlear hydrops preferentially affects the apex of the cochlea where low-frequency sounds are interpreted. Due to the fluid imbalance in this area, parts of the cochlea are stretched or under more tension than usual, which can lead to distortions of sound, changes in pitch perception, or hearing loss, all usually in the low frequencies. Common symptoms include:

Low-frequency sensorineural hearing loss. A feeling of fullness in the ear. Tinnitus (ringing in the ear). Diplacusis (the perception of sound being a different pitch in one ear). Hyperacusis (an intolerance to loud sounds).
Sialuria is a group of disorders resulting in an accumulation of free sialic acid. One type, known as the Finnish type or Salla disease has been described in northeastern Finland and is due to a mutation in gene SLC17A5 on chromosome 6q4-15. The "French type sialuria" (Online Mendelian Inheritance in Man (OMIM): 269921), is a very rare condition presenting in infancy with failure to thrive, yellowish skin, large liver, low blood count, recurrent chest infections, bowel upsets, dehydration and characteristic facial features. References


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