Diagnoses

By Marcello Cherchi, MD PhD

The following outline is roughly organized anatomically, categorizing diseases into those predominantly due to dysfunction of the ear (“otologic”), brain (“neurologic”), eye (“ophthalmologic”), heart (“cardiovascular”), general medical problems, multiple causes, and audio-vestibular symptoms arising in the context of other diseases.

You can also find a disease using the search function, which will scan the entire website.

You can also use our preliminary diagnostic algorithm for disequilibrium.

1. Otologic

   1. Diseases of the labyrinth

      1. ✅ Benign paroxysmal positional vertigo (BPPV) in general
         1. ✅ Posterior canal BPPV
         2. ✅ Lateral canal BPPV
         3. ✅ Anterior canal BPPV
      2. Endolymphatic hydrops and related phenomena
         1. ✅ Meniere’s disease
         2. ✅ Otolithic catastrophe of Tumarkin
         3. ✅ Post-traumatic endolymphatic hydrops
      3. Immune-mediated phenomena
         1. ✅ Autoimmune inner ear disease (AIED)
         2. ✅ Sympathetic ear disease
      4. Noise-induced and pressure-induced otologic damage
         1. ✅ Noise induced hearing loss
         2. ✅ Noise induced vestibular damage
         3. ✅ Otic barotrauma
      5. ✅ Presbycusis (age-related hearing loss)
      6. ✅ Ototoxicity
      7. ✅ Inner ear ischemia
      8. ✅ Labyrinthine ossification
      9. ✅ Audio-vestibular symptoms following cochlear implantation

   2. Diseases involving the vestibulocochlear nerve

      1. ✅ Vestibular, cochlear, and vestibulocochlear paroxysmia
      2. ✅ Vestibular schwannoma
      3. ✅ Auditory neuropathy
      4. ✅ Ramsay Hunt syndrome (herpes zoster oticus)
      5. ✅ Multiple myeloma

   3. Diseases involving the middle ear

      1. ✅ Middle ear myoclonus (tensor tympani myoclonus, stapedial myoclonus)
      2. ✅ Eustachian tube dysfunction (ETD)
      3. ✅ Otitis media and middle ear effusion
      4. ✅ Cholesteatoma
      5. ✅ Glomus tympanicum

   4. Diseases potentially involving multiple structures

      1. Vestibular weakness
         1. ✅ Vestibular weakness in general
         2. ✅ Bilateral vestibular weakness (BVW)
      2. ✅ Lindsay-Hemenway syndrome
      3. ✅ Vestibular neuritis, labyrinthitis and sudden sensorineural hearing loss
         1. ✅ Vestibular neuritis
         2. ✅ Labyrinthitis
         3. ✅ Sudden sensorineural hearing loss (SSNHL)
         4. ✅ A continuum of vestibular neuritis, labyrinthitis and sudden sensorineural hearing loss?
      4. Conditions involving reduced labyrinthine resistance
         1. ✅ Third window phenomena
         2. ✅ Semicircular canal dehiscence (SCD)
         3. ✅ Perilymphatic fistula (PLF)
      5. Tinnitus
         1. ✅ Idiopathic tinnitus
         2. ✅ Exploding head syndrome
         3. Somatic tinnitus
            1. ✅ Pulsatile tinnitus
            2. ✅ Tinnitus related to temporomandibular joint dysfunction
            3. ✅ Tinnitus related to the neck (cervicogenic tinnitus)
      6. Diseases of bone
         1. ✅ Otosclerosis
         2. ✅ Paget’s disease of bone (osteitis deformans)
         3. ✅ Fibrous dysplasia
      7. Trauma
         1. ✅ Labyrinthine concussion
         2. ✅ Temporal bone fracture
         3. ✅ Stapes luxation/subluxation, and prosthetic stapes protrusion/migration
      8. ✅ Hyperacusis
      9. ✅ Otalgia and referred otalgia

   5. Congenital otologic malformations

      1. ✅ Large vestibular aqueduct
      2. ✅ Large cochlear aqueduct
      3. ✅ Mondini dysplasia
      4. ✅ Pendred syndrome
      5. ✅ Usher syndrome

2. Neurologic:

   1. Brain
      1. Migraine and its possible variants
         1. ✅ Migraine
         2. ✅ Migraine associated vertigo (MAV), also called vestibular migraine (VM)
         3. ✅ Cyclic vomiting syndrome
      2. Sensitivity to motion and to illusions of motion
         1. ✅ Motion sickness (constitutional motion sensitivity)
         2. ✅ Cybersickness
         3. ✅ Motorist disorientation syndrome (MDS)
      3. Internal models
         1. ✅ Mal de debarquement syndrome (MdDs)
         2. ✅ Persistent postural perceptual dizziness (PPPD)
      4. ✅ Central positional nystagmus (CPN)
      5. ✅ Concussion and post-concussive disequilibrium
      6. ✅ White matter disease and disequilibrium
      7. ✅ Stroke and disequilibrium
      8. ✅ Encephalitis
      9. ✅ Epileptic vertigo
      10. ✅ Superficial siderosis
   2. Spinal cord
      1. ✅ Cervicogenic vertigo (CV)
      2. ✅ Spinal cord disorders
   3. Peripheral neuropathy
      1. ✅ Peripheral neuropathy in general
      2. ✅ Charcot-Marie-Tooth (CMT) disease
      3. ✅ Guillain-Barre syndrome (GBS) and other acute inflammatory demyelinating polyradiculoneuropathies (AIDPs)
      4. ✅ Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)
   4. Multiple structure syndromes
      1. ✅ Amyotrophic lateral sclerosis (ALS)
      2. ✅ CANVAS (cerebellar ataxia, neuropathy, vestibular areflexia syndrome)
      3. ✅ Refsum disease (RD)
      4. ✅ Susac syndrome
      5. ✅ Wolfram syndrome
   5. Ataxias
      1. ✅ General discussion of ataxias
      2. ✅ Spinocerebellar ataxias and other genetic ataxias
      3. ✅ Episodic ataxias and acetazolamide responsive ataxias
      4. ✅ Gluten ataxia
      5. ✅ Friedreich ataxia
   6. Movement disorders
      1. ✅ Parkinsonism
      2. ✅ Idiopathic Parkinson’s disease
      3. ✅ Progressive supranuclear palsy (PSP)
      4. ✅ Multiple system atrophy (MSA)
      5. ✅ Huntington’s disease
      6. ✅ Stiff person syndrome (SPS)
      7. ✅ Fahr disease (FD)
   7. Posterior fossa lesions
      1. ✅ Chiari malformations
      2. ✅ Basilar invagination
      3. ✅ Paraneoplastic syndrome
   8. Intracranial pressure disorders
      1. ✅ Hydrocephalus with increased intracranial pressure
      2. ✅ Normal pressure hydrocephalus (NPH) 
      3. ✅ Intracranial pressure fluctuations and hearing loss
   9. Neurologically-mediated visual disorders
      1. ✅ Visual snow syndrome (VSS)
      2. ✅ Misokinesia
   10. Neurologically-mediated auditory disorders
       1. ✅ Cortical deafness
       2. ✅ Misophonia
       3. ✅ Musical hallucinations
   11. Specific ocular motor syndromes
       1. ✅ Centripetal nystagmus
       2. ✅ Congenital nystagmus
       3. ✅ Convergence-retraction nystagmus
       4. ✅ Internuclear ophthalmoplegia (INO)
       5. ✅ Latent nystagmus
       6. ✅ Ocular flutter
       7. ✅ Opsoclonus and the opsoclonus myoclonus syndrome
       8. ✅ Palatal and oculopalatal tremor
       9. ✅ Periodic alternating nystagmus (PAN)
       10. ✅ Rebound nystagmus
       11. ✅ See-saw nystagmus and hemi-see-saw nystagmus
       12. ✅ Spontaneous down beat nystagmus 
       13. ✅ Spontaneous up beat nystagmus
       14. ✅ Square wave jerks
       15. ✅ Staircase saccades
       16. ✅ Windmill nystagmus
   12. Eye movements elicited by specific provocative maneuvers
       1. ✅ Head-shaking nystagmus
       2. ✅ Hyperventilation-induced nystagmus
       3. ✅ Valsalva-induced nystagmus
       4. ✅ Vibration-induced nystagmus
   13. ✅ Neurologic disease processes that can involve any part of the central nervous system (infarction, demyelination, space-occupying lesions)
   14. ✅ Selected vascular phenomena in otoneurology
   15. Intersections between audio-vestibular disease, psychology and psychiatry
       1. Interface between otoneurology and psychology
          1. ✅ Psychological disequilibrium
          2. ✅ Dissociative experiences in vestibular disorders
          3. ✅ Psychiatric disease and vestibular dysfunction
       2. The relationship between cognitive deficits and audio-vestibular disease
          1. ✅ Can disequilibrium cause cognitive impairment?
          2. ✅ Can cognitive impairment cause disequilibrium?
          3. ✅ Can hearing loss cause cognitive impairment?
          4. ✅ Can tinnitus cause cognitive impairment?
          5. ✅ “Brain fog”

3. Ophthalmologic and visual factors in disequilibrium

   1. ✅ Visual vertigo (VV)
   2. ✅ Ophthalmological visual deficits and disequilibrium
   3. ✅ Superior oblique myokymia
   4. ✅ Illusions of inversion and tilt

4. Cardiovascular:

   1. Orthostatic intolerance
      1. ✅ Orthostatic hypotension and postural orthostatic tachycardia
      2. ✅ Orthostatic hypertension
   2. ✅ Cardiac arrhythmia
   3. ✅ Vestibular syncope

5. Medical

   1. ✅ Pharmacologic causes of disequilibrium
   2. ✅ Postprandial disequilibrium
   3. Infectious
      1. ✅ COVID-19
      2. ✅ Epstein-Barr virus
      3. ✅ Human immunodeficiency virus (HIV) and audio-vestibular symptoms
      4. ✅ Lyme disease
      5. ✅ Syphilis (otosyphilis)
      6. ✅ West Nile virus
   4. Endocrine, nutrition and metabolism
      1. ✅ Thyroid disease and disequilibrium
      2. ✅ Vitamin B1 (thiamine) deficiency
      3. ✅ Vitamin B12 (cyanocobalamin) deficiency
      4. ✅ Vitamin D deficiency
      5. ✅ Wilson’s disease
      6. ✅ Zinc deficiency
      7. ✅ Altitude sickness
   5. Toxic
      1. ✅ Alcohol
      2. ✅ Caffeine
      3. ✅ Carbon monoxide
      4. ✅ Cocaine
      5. ✅ Heavy metals
      6. ✅ Jet propulsion type 8 fuel
      7. ✅ Marijuana (cannabis)
      8. ✅ MDMA, “ecstasy”
      9. ✅ Nicotine and tobacco
      10. ✅ Phencyclidine (PCP)
   6. Electromagnetic fields and radiation
      1. ✅ Radiation and its otovestibular effects
      2. ✅ Electromagnetic fields (EMFs)

6. Presentations with multiple potential causes

   1. ✅ Multifactorial disequilibrium
   2. ✅ Presbyvestibulopathy
   3. ✅ Drop attacks
   4. ✅ Coital vertigo

7. Audio-vestibular symptoms occurring in the context of other conditions

   1. ✅ Chronic fatigue syndrome (CFS) and audio-vestibular symptoms
   2. ✅ Fibromyalgia and audio-vestibular symptoms
   3. ✅ Ehlers-Danlos syndrome (EDS) and audio-vestibular symptoms
   4. ✅ Pregnancy, delivery and audio-vestibular symptoms
   5. ✅ Mitochondrial disorders and audio-vestibular symptoms
   6. ✅ Immunologically-mediated disorders and the audio-vestibular system
      1. ✅ Allergy and audio-vestibular symptoms
      2. ✅ Behcet disease and audio-vestibular symptoms
      3. ✅ Cogan syndrome and audio-vestibular symptoms
      4. ✅ Granulomatosis with polyangiitis (Wegener’s granulomatosis) and audio-vestibular symptoms
      5. ✅ Lupus (systemic lupus erythematous) and audio-vestibular symptoms
      6. ✅ Rheumatoid arthritis (RA) and audio-vestibular symptoms
      7. ✅ Sarcoidosis and audio-vestibular symptoms
      8. ✅ Scleroderma and audio-vestibular symptoms
      9. ✅ Sjogren syndrome and audio-vestibular symptoms
      10. ✅ Vogt-Koyanagi-Harada syndrome and audio-vestibular symptoms