Chapter 8

Ear, Nose, and Throat Manifestations

of Sjögren’s Syndrome

Rohan R. Walvekar and Francis Marchal

8.1Introduction

Sjögren’s syndrome (SjS) is an autoimmune disease that primarily affects the secretory function of lacrimal and salivary glands [1]. Dry mouth (xerostomia) is the most common presenting complaint [2], but SjS can affect multiple organs including the kidneys, lungs, liver, blood vessels, and lymph nodes, resulting in a myriad of clinical manifestations [3]. This chapter provides an overview of the key manifestations of SjS associated with the ear, nose, and throat region. We review the otologic, sinus and nasal, laryngopharyngeal, and tracheal manifestations of this disease.

8.2Otologic Manifestations

The otologic manifestations associated with SjS have not been extensively reported but are generally mild [4]. Most otologic complaints consist of hearing loss (25%), which is often a mild to moderate sensorineural hearing loss in the high frequencies

R.R. Walvekar

Department of Otolaryngology Head and Neck Surgery, LSU Health Sciences Center, Salivary Endoscopy Service and Clinical Research, New Orleans, LA, USA

F. Marchal (*)

Department of Otolaryngology Head and Neck Surgery, Sialendoscopy Unit, European Sialendoscopy Training Center (ETSC), University Hospital of Geneva, Geneva, Switzerland

[3–5]. Although the pathophysiology of this hearing loss is not well understood, it is postulated to be due to the deposition of immune complexes in the inner ear stria vascularis and subsequent ischemia of the inner ear arterial microvasculature. Autoantibodies to cardiolipin and M3 muscarinic receptors in patient sera are suspected to play a role in the pathogenesis of this hearing loss [3]. Other proposed theories include autoantibodies to ciliar epitopes in patients with increased genetic susceptibility or increased production of gamma interferon and inflammatory cytokines by inner ear cells [3, 6–8].

The deposition of the immune complexes occurs at the basal turn of the cochlea, which represents hearing in the high frequencies [4]. Consequently, patients with SjS have high frequency sensorineural hearing loss that can be either unilateral or bilateral [4]. Studies evaluating otologic symptoms in patients with SjS suggest that the prevalence of hearing loss is lower in patients with primary SjS (4.5–27%) as compared to those with secondary SjS (19.4–46%) [1, 4]. This is consistent with the notion that sensorineural hearing loss is associated with systemic autoimmune disease rather than SjS per se. Current evidence is still insufficient to confirm this hypothesis. Mild to severe sensorineural hearing loss may also be an early manifestation of SjS [3] and consequently could be included in the workup of patients with sicca complex.

Patients with SjS can also present with complaints related to dryness of the external auditory canal skin, otalgia or ear pain, tinnitus, vertigo, and recurrent ear infections. Hearing loss, otalgia, and tinnitus have been reported to occur in 25% of patients presenting with otologic symptoms. These symptoms are proposed to be due to crust or lymphoid masses obstructing the eustachian tube orifice [9].

Doig et al. [1] were the first to report audiological findings in SjS. The study evaluated 22 patients with primary SjS, 31 with secondary SjS (rheumatoid arthritis and SjS), and 21 with RA. Evaluations included a comprehensive otologic history with examination of the ears and audiometry. Audiometric evaluation were graded 1 (normal hearing), 2 (maximum limit of hearing loss acceptable as normal), and 3 (impaired hearing both conductive and sensorineural). The authors reported otalgia and tinnitus in 4.5% (1 of 22) and dryness of the external auditory canal, dry wax, or/and abnormal/perforated ear drum in 9.1% (2 of 22) patients with primary SjS. A low incidence of otologic symptoms was recorded for patients with secondary SjS, as well. However, Campbell et al. [9] reported that dryness of the external canal is more frequent than abnormalities of the tympanic membrane.

Doig et al. also suggested that patients with SjS are at risk for otitis media with effusion. However, in a more recent study, Freeman et al. evaluated 196 patients with SjS including 60 patients with unclassified sicca syndrome (according to the revised 2002 international classification criteria [10]) and did not report an increased prevalence of middle ear pathology or risk for otitis media with effusion [4].

Although the otologic manifestations of SjS are not frequent, they can cause significant quality-of-life impairment. It would be ideal to include a comprehensive otologic workup including a microscopic ear examination, review of otologic symptoms, and objective hearing evaluation (speech audiometry, auditory brainstemevoked responses, and impedance audiometry) to evaluate external, middle, and inner ear pathology as well as rule out retrocochlear and brainstem lesion that can be associated with sensorineural hearing loss.

nasal dryness, crusting, congestion, hyposmia, and epistaxis. Freeman et al. [4] reported nasal symptoms in up to 50% of patients in their study. However, nasal examination with anterior rhinoscopy revealed abnormal findings in only 20% [4]. Nasal dryness has been reported from 18% to 73% in various studies [1, 9, 12, 13], and crusting in 18.5% [1], 44% [12], and up to 50% [9] in different studies. Hyposmia or a decreased sense of smell is present in 30–45% of patients and is often associated with hypoguesia [1, 5, 9, 14]. Chronic sinusitis has also been reported in patients with SjS [5].

Epistaxis is commonly reported in patients with SjS. Doig et al. reported epistaxis in 31% of patients with primary SjS [1]. However, Freeman and colleagues reported a divergent experience [4]. In fact, while Doig et al. and other studies have reported objective findings of nasal crusting, nasal atrophy, and nasal dryness in up to 54% of patients, Freeman and colleagues found abnormal nasal examinations in only 20% of their study population [1, 4]. These differences in presenting symptoms and clinical findings can be explained by individual patient variability, small sample sizes, and geographical variability in study populations.

Although, nasal symptoms are prevalent in patients with SjS, they are often not dominant presenting symptoms. Consequently, it is incumbent on the physician to question patients regarding these symptoms and to perform an evaluation of nasal symptoms and a complete nasal examination, including anterior rhinoscopy and nasal endoscopic evaluation of the nasal cavity and nasopharynx. Smell disturbances can be further quantified utilizing test such as the UPSIT (University of Pennsylvania Smell Identification Test) test (Sensonics Inc, Haddon Heights, NJ). The UPSIT test is a standardized microcapsulated odorant test that can be applied over 15–20 min on an outpatient basis. Scores range from 19 to 40, categorizing patients from severe microsmia to normal smell appreciation [15, 16].

8.4Laryngopharyngeal and Tracheal Manifestations

The symptom of dry mouth and physical findings consistent with xerostomia are hallmarks of SjS and have been reported in up to 100% and 94% of patients, respectively [1, 9, 17–21]. The oral manifestations of SjS are comprehensively described in Chap. 6. However, it is important to reiterate that dry mouth and xerostomia are intimately related to and often contribute to symptoms and manifestations within the larynx, pharynx, and trachea. Laryngopharyngeal symptoms generally occur with or after the onset of xerostomia [14].

Pharyngeal involvement usually is related to absence of saliva and dryness of the pharyngeal mucosa [9]. Thick, tenacious secretions can also contribute to these symptoms. Most patients complain of food sticking to the mouth, difficulty swallowing food, the need to drink water to wash food down the esophagus, and avoidance of dry foods [9]. Patients may also complain of dry cough and a foreign body or globus sensation in the throat.

Freeman et al. [4] reported that abnormal findings in the throat and larynx were detected in only 24% of patients, despite the fact that approximately 80% of the

Fig. 8.2 Bamboo node: middle third right vocal cord lesion (black arrow points to nodule)

patients in their study had laryngopharyngeal complaints. Previous studies have shown that laryngopharyngeal symptoms are often associated with significant esophageal dysmotility (10%) [22, 23], the presence of esophageal webs, and postcricoid narrowing [9]. Consequently, throat and laryngeal complaints must be taken seriously and investigated appropriately. A detailed review of esophageal manifestations of SjS is presented in Chap. 16.

Hoarseness and dsyphonia are late manifestations of SjS. Bloch et al. reported hoarseness in 20 (32%) of their 62 patients with primary SjS [24]. Hoarseness is usually related to chronic mucosal dryness or tenacious mucus that coats the vocal cords. Other causes in include cricoarytenoid joint arthritis, laryngitis, granulomatous or nongranulomatous nodules, laryngeal mucosal thickening, or recurrent laryngeal nerve paresis or palsy [25]. Movement of the vocal cords is usually normal on examination, suggesting that cricoarytenoid joint involvement as seen in RA is uncommon in SjS [1]. However, a vocal fold lesion can be the presenting symptom of SjS [5]. In addition, a vocal cord lesion unique to autoimmune diseases known as the “bamboo node” has been described in SjS. The lesion is a white or yellow transverse submucosal lesion typically located in the middle third of the vocal fold (Fig. 8.2) [26]. Murano et al. reported two cases of bamboo nodes in patients with SjS [27].

Granulomatous and nongranulomatous nodules have also been described in SjS. Granulomatous nodules have no site predilection. In contrast, nongranulomatous nodules are symmetrical and characteristically involve the anterior third of the true cords. They are similar in appearance and histology to vocal nodules secondary to vocal abuse [5, 25]. Patients may not have visible laryngeal lesions but can present with hoarseness. Clinical and experimental data suggest that this could be explained by submucosal deposition of immune complexes that may be present for long durations of time prior to development of visible lesions. Gilliam and Cheatum examined the basement membrane of a patient with skin and laryngeal lesion with systemic lupus erythematosus and demonstrated immune complexes that confirmed similar postulations by Cooke in 1972 [25, 28, 29].