By Marcello Cherchi, MD PhD
For patients
Lyme disease happens when a person is infected by a bacterium called Borrelia burgdorferi. Some patients with Lyme disease may get ear symptoms (pain, hearing loss, ringing in the ears) and disequilibrium.
For clinicians
Practical summary
Lyme disease results from infection by Borrelia burgdorferi. It can present in many ways, including with multiple cranial neuropathies. From the otovestibular perspective, it can manifest with various combinations of symptoms referrable to the vestibulocochlear nerve, including any combination of hearing loss, tinnitus, otalgia, and disequilibrium. Otovestibular testing may show sensorineural hearing loss and caloric weakness.
Introduction
Borrelia burgdorferi is a tick-borne spirochete bacterial infection that causes Lyme disease in humans. Lyme disease sometimes presents in classical fashion with a “bullseye rash,” and can develop a broad range of systemic and organ-specific manifestations, including multiple cranial neuropathies. Here we limit the discussion to otovestibular manifestations of this disease.
Epidemiology
The epidemiology and geographic distribution of Lyme disease has been well described (Mead 2015).
Pathophysiological mechanism of disease
Despite considerable research, there remains uncertainty about the pathophysiological mechanism by which Lyme disease causes symptoms (Donta et al. 2021).
Clinical presentation
Lyme disease can also present with multiple symptoms referrable to the vestibulocochlear nerve, including hearing loss, tinnitus, disequilibrium and otalgia (Goldfarb and Sataloff 1994; Moscatello et al. 1991), or some subset of those symptoms (Vos et al. 2016). For example, Lyme disease has been reported to present (initially) with only sudden hearing loss (Espiney Amaro et al. 2015; Peeters et al. 2013); in the late stage of infection, it has been reported to present solely with disequilibrium (Jozefowicz-Korczynska et al. 2019; Peltomaa et al. 1998).
One study analyzed 38 patients (11 male, 27 female) with serologically confirmed Lyme disease, all of whom complained of disequilibrium. Of these 38 patients, 11 (29%) had also experienced some form of hearing loss, 2 (5%) specifically had sudden hearing loss, and 29 (76%) complained of tinnitus.
Sowula and colleagues (Sowula et al. 2021) recruited 50 patients (35 women, 15 men) with serologically confirmed Lyme disease and studied them with audiometry, auditory brainstem evoked responses and videonystagmography. Of these 50 patients, 38 (76%) reported symptoms of “vertigo” and 29 (76%) complained of tinnitus. Of the 38 patients who complained of vertigo, 11 (29%) experienced hearing loss. Of those 11 patients with hearing loss, the hearing loss was bilateral in two, and presented with sudden onset in two. The investigators reported there to be a statistically significant association between the ”degree of labyrinth damage” (as measured by caloric weakness) and the presence of high frequency hearing loss (not low frequency hearing loss).
Testing: vestibular
One study compared 91 patients with Ménière’s disease and 11 patients with serologically confirmed Lyme disease. Of the 11 patients with Lyme disease, 6 (55%) had symptoms of sensorineural hearing loss, tinnitus and disequilibrium — in other words, the phenotype of Ménière’s disease, and their workup also showed similar results (such as endolymphatic hydrops on electrocochleography) (Selmani et al. 2002). However, as noted earlier in this discussion, another study (Sowula et al. 2021) reported that patients with Lyme disease complaining of vertigo have high frequency hearing loss (rather than the low frequency hearing loss that would be more typical of Ménière’s disease).
Testing: other
One study of 47 consecutive patients with sudden hearing loss (and no other symptoms) reported that 21% of the cases were found to have positive Lyme titers, though there was no difference in the clinical course of the seropositive and seronegative patients (Lorenzi et al. 2003).
Imaging
Brain MRI in patients with Lyme disease may demonstrate enhancement of cranial nerves, including the vestibulocochlear nerve (Piche-Renaud et al. 2018).
Differential diagnosis
For each otovestibular symptom that Lyme disease causes there is a broad differential diagnosis. It should be kept in mind that other tick-borne illnesses (besides Lyme disease) can manifest with otovestibular symptoms (Sowula et al. 2018).
Treatment
Lyme disease is usually treated with doxycycline, though specific management may depend on the particular organ system involvement.
References
Donta ST, States LJ, Adams WA, Bankhead T, Baumgarth N, Embers ME, Lochhead RB, Stevenson B (2021) Report of the Pathogenesis and Pathophysiology of Lyme Disease Subcommittee of the HHS Tick Borne Disease Working Group. Front Med (Lausanne) 8: 643235. doi: 10.3389/fmed.2021.643235
Espiney Amaro C, Montalvao P, Huins C, Saraiva J (2015) Lyme disease: sudden hearing loss as the sole presentation. J Laryngol Otol 129: 183-6. doi: 10.1017/S0022215114003417
Goldfarb D, Sataloff RT (1994) Lyme disease: a review for the otolaryngologist. Ear Nose Throat J 73: 824-9.
Jozefowicz-Korczynska M, Zamyslowska-Szmytke E, Piekarska A, Rosiak O (2019) Vertigo and Severe Balance Instability as Symptoms of Lyme Disease-Literature Review and Case Report. Front Neurol 10: 1172. doi: 10.3389/fneur.2019.01172
Lorenzi MC, Bittar RS, Pedalini ME, Zerati F, Yoshinari NH, Bento RF (2003) Sudden deafness and Lyme disease. Laryngoscope 113: 312-5. doi: 10.1097/00005537-200302000-00021
Mead PS (2015) Epidemiology of Lyme disease. Infect Dis Clin North Am 29: 187-210. doi: 10.1016/j.idc.2015.02.010
Moscatello AL, Worden DL, Nadelman RB, Wormser G, Lucente F (1991) Otolaryngologic aspects of Lyme disease. Laryngoscope 101: 592-5. doi: 10.1288/00005537-199106000-00004
Peeters N, van der Kolk BY, Thijsen SF, Colnot DR (2013) Lyme disease associated with sudden sensorineural hearing loss: case report and literature review. Otol Neurotol 34: 832-7. doi: 10.1097/MAO.0b013e31827c9f93
Peltomaa M, Pyykko I, Seppala I, Viljanen M (1998) Lyme borreliosis–an unusual cause of vertigo. Auris Nasus Larynx 25: 233-42. doi: 10.1016/s0385-8146(97)10039-6
Piche-Renaud PP, Branson H, Yeh EA, Morris SK (2018) Lyme disease presenting with multiple cranial neuropathies on MRI. IDCases 12: 117-118. doi: 10.1016/j.idcr.2018.04.004
Selmani Z, Pyykko I, Ishizaki H, Ashammakhi N (2002) Use of electrocochleography for assessing endolymphatic hydrops in patients with Lyme disease and Meniere’s disease. Acta Otolaryngol 122: 173-8. doi: 10.1080/00016480252814180
Sowula K, Skladzien J, Szaleniec J, Gawlik J (2018) Otolaryngological symptoms in patients treated for tick-borne diseases. Otolaryngol Pol 72: 30-34. doi: 10.5604/01.3001.0011.5948
Sowula K, Szaleniec J, Dworak M, Przeklasa M, Maraj M, Ceranowicz P, Tomik J (2021) Vertigo as One of the Symptoms of Lyme Disease. J Clin Med 10. doi: 10.3390/jcm10132814
Vos FI, Merkus P, van Nieuwkerk EB, Hensen EF (2016) Rare cause of bilateral sudden deafness. BMJ Case Rep 2016. doi: 10.1136/bcr-2016-216004
